Kawasaki disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2] Arzu Kalayci, M.D. [3]

Overview

Kawasaki disease, also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin and mucous membranes, lymph nodes, blood vessel walls, and the heart. It does not seem to be contagious. It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. Additional risk factors in the United States include Asian race and male sex. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. Common symptoms of kawasaki disease include high grade fever, red eyes, bright red and cracked lips, red mucous membranes in the mouth, strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue, red palms of the hands and the soles of the feet, swollen hands and feet, and rash. Intravenous Immunoglobulin (IVIG) and aspirin are indicated in kawasaki disease.

Historical Perspective

The Historical data on Kawasaki is described below:

• In 1961, Dr. Tomisaku Kawasaki saw his first case of Kawasaki disease.
• In 1967, Kawasaki published his first report of Kawasaki disease in Japanese.
• In 1960s, pathologist Noboru Tanaka and pediatrician Takajiro Yamamoto disputed the early assertion of Kawasaki that Kawasaki disease was a self-limited illness with no sequelae.
• In 1970, first Japanese nationwide survey of Kawasaki disease was conducted and 10 autopsy cases of sudden cardiac death after Kawasaki disease were documented.
• In 1973, at the University of Hawaii hospital, pathologist Eunice Larson, in collaboration with Benjamin Landing at the Los Angeles Children's Hospital, retrospectively established a diagnosis of Kawasaki disease in a 1971 autopsy case.
• In 1974, Tomisaku Kawasaki published the first English-language report of 50 patients with Kawasaki disease.
• By 1974, the link between Kawasaki disease and coronary artery vasuclitis was well established.
• In 1976, he first cases of Kawasaki disease outside of Japan were reported in Hawaii.
• In 1988, the Committee on infectious diseases of the American Academy of Pediatrics declared IVIG treatment as the recommended therapy for Kawasaki disease.
• In 2006 march, Kawasaki disease was mentioned in the television programs Nip/Tuck and Without a Trace In the episode All In of the TV series House, it was inexplicably mentioned as a possible diagnosis for a 6 year old boy that was admitted with bloody diarrhea and coordination problems, as well as an elderly woman with unexplained respiratory, cardiovascular and neural deficiencies. Maxie Jones, a fictional character on General Hospital suffers from it. According to John Travolta and Kelly Preston, their son Jett Travolta also suffers from the disease.

Classification

There is no established system for the classification of Kawasaki disease.

Pathophysiology

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR

[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Causes

The causative agent of Kawasaki disease is still unknown. However, current etiological theories center primarily on immunological causes for the disease. Much research is being performed to discover a definitive toxin or antigenic substance, possibly a superantigen, that is the specific cause of the disease. An unknown virus may play a role as an inciting factor as well.

Differentiating Kawasaki disease from other diseases

Different rash-like conditions can be confused with Kawasaki disease and are thus included in its differential diagnosis. The various conditions that should be differentiated from Kawasaki disease include:

Disease	Features
Kawasaki disease	Commonly presents with high and persistent fever, red mucous membranes in mouth, "strawberry tongue", swollen lymph nodes and skin rash in early disease, with peeling off of the skin of the hands, feet and genital area.
Impetigo	It commonly presents with pimple-like lesions surrounded by erythematous skin. Lesions are pustules, filled with pus, which then break down over 4-6 days and form a thick crust. It's often associated with insect bites, cuts, and other forms of trauma to the skin.
Insect bites	The insect injects formic acid, which can cause an immediate skin reaction often resulting in a rash and swelling in the injured area, often with formation of vesicles.
Measles	Commonly presents with high fever, coryza and conjunctivitis, with observation of oral mucosal lesions (Koplik's spots), followed by widespread skin rash.
Monkeypox	The presentation is similar to smallpox, although it is often a milder form, with fever, headache, myalgia, back pain, swollen lymph nodes, a general feeling of discomfort, and exhaustion. Within 1 to 3 days (sometimes longer) after the appearance of fever, the patient develops a papular rash, often first on the face. The lesions usually develop through several stages before crusting and falling off.
Rubella	Commonly presents with a facial rash which then spreads to the trunk and limbs, fading after 3 days, low grade fever, swollen glands, joint pains, headache and conjunctivitis. The rash disappears after a few days with no staining or peeling of the skin. Forchheimer's sign occurs in 20% of cases, and is characterized by small, red papules on the area of the soft palate.
Atypical measles	The symptoms commonly begin about 7-14 days after infection and present as fever, cough, coryza and conjunctivitis. Observation of Koplik's spots is also a characteristic finding in measles.
Coxsackievirus	The most commonly caused disease is the Coxsackie A disease, presenting as hand, foot and mouth disease. It may be asymptomatic or cause mild symptoms, or it may produce fever and painful blisters in the mouth (herpangina), on the palms and fingers of the hand, or on the soles of the feet. There can also be blisters in the throat or above the tonsils. Adults can also be affected. The rash, which can appear several days after high temperature and painful sore throat, can be itchy and painful, especially on the hands/fingers and bottom of feet.
Acne	It is typical of teenagers, usually appears on the face and upper neck, but the chest, back and shoulders may have acne as well. The upper arms can also have acne, but lesions found there are often keratosis pilaris, not acne. The typical acne lesions are comedones and inflammatory papules, pustules, and nodules. Some of the large nodules were previously called "cysts"
Syphilis	It commonly presents with gneralized systemic symptoms such as malaise, fatigue, headache and fever. Skin eruptions may be subtle and asymptomatic It is classically described as: Non-pruritic bilateral symmetrical mucocutaneous rash Non-tender regional lymphadenopathy Condylomata lata and Patchy alopecia.
Molluscum contagiosum	The lesions are commonly flesh-colored, dome-shaped, and pearly in appearance. They are often 1-5 millimeters in diameter, with a dimpled center. Generally not painful, but they may itch or become irritated. Picking or scratching the lesions may lead to further infection or scarring. In about 10% of the cases, eczema develops around the lesions. They may occasionally be complicated by secondary bacterial infections.
Mononucleosis	Common symptoms include low-grade fever without chills, sore throat, white patches on tonsils and back of the throat, muscle weakness and sometime extreme fatigue, tender lymphadenopathy, petechial hemorrhage and skin rash.
Toxic erythema	It is a common rash in infants, with clustered and vesicular appearance.
Rat-bite fever	It commonly presents with fever, chills, open sore at the site of the bite and rash, which may show red or purple plaques.
Parvovirus B19	The rash of fifth disease is typically described as "slapped cheeks," with erythema across the cheeks and sparing the nasolabial folds, forehead, and mouth.
Cytomegalovirus	The common symptoms include sore throat, swollen lymph nodes, fever, headache, fatigue, weakness, muscle pain and loss of appetite.
Scarlet fever	It commonly includes fever, punctate red macules on the hard and soft palate and uvula (Forchheimer's spots), bright red tongue with a "strawberry" appearance, sore throat and headache and lymphadenopathy.
Rocky Mountain spotted fever	The symptoms may include maculopapular rash, petechial rash, abdominal pain and joint pain.
Stevens-Johnson syndrome	The symptoms may include fever, sore throat and fatigue. Commonly presents ulcers and other lesions in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children. A rash of round lesions about an inch across, may arise on the face, trunk, arms and legs, and soles of the feet, but usually not on the scalp.
Varicella-zoster virus	It commonly starts as a painful rash on one side of the face or body. The rash forms blisters that typically scab over in 7-10 days and clears up within 2-4 weeks.
Chickenpox	It commonly starts with conjunctival and catarrhal symptoms and then characteristic spots appearing in two or three waves, mainly on the body and head, rather than the hands, becoming itchy raw pox (small open sores which heal mostly without scarring). Touching the fluid from a chickenpox blister can also spread the disease.
Meningococcemia	It commonly presents with rash, petechiae, headache, confusion, and stiff neck, high fever, mental status changes, nausea and vomiting.
Rickettsial pox	The first symptom is commonly a bump formed by a mite-bite, eventually resulting in a black, crusty scab. Many of the symptoms are flu-like including fever, chills, weakness and muscle pain but the most distinctive symptom is the rash that breaks out, spanning the person's entire body.
Meningitis	It commonly presents with headache, nuchal rigidity, fever, petechiae and altered mental status.

Disease	Epidemiology	Predisposing factors	Clinical features		Lab abnormalities
			Signs	Symptoms
Kawasaki disease	Occurs in children, usually age 1-4 years	Interaction of genetic and environmental factors, possibly including an infection in combination with genetic predisposition to an autoimmune mechanism (autoimmune vasculitis)	Non-suppurative, painless bilateral conjunctival inflammation (conjunctivitis), strawberry tongue (marked redness with prominent gustative papillae), deep transverse grooves across the nails may develop (Beau’s lines), lymphadenopathy present(acute, non-purulent, cervical), may lead to coronary artery aneurysms.	High and persistent fever that is not very responsive to normal treatment with acetaminophen or NSAIDs,  diffuse macular-papular erythematous rash	Liver function tests may show evidence of hepatic inflammation and low serum albumin levels, low hemoglobulin and age-adjusted hemoglobulin concentrations, thrombocytosis, anemia. Echocardiographic abnormalities, such as valvulitis (mitral or tricuspid regurgitation) and coronary artery lesions, are significantly more common in Kawasaki disease. Pyuria of uretheral origin.
Toxic shock syndrome	Occurs in both adults and children (9:1 female predominance)	Occurs in association with vaginitis during menstruation following tampon use (S. aureus); as a complication of soft tissue infections (S. pyogenes or GAS) or in females undergoing medical abortion (C. sordellii).	Hypotension, tachycardia, mucous membrane hyperemia (vaginal, oral, conjunctival)	Fever, diarrhea, vomiting, diffuse scarlantiform rash	Hyponatremia and uremia. Hepatic dysfunction (total bilirubin, serum asparate aminotransferase or serum alanine aminotransferase levels >2 times upper normal limit), leukocytosis with a polymorphonuclear shift to the left. Platelets < 100,000 per mm3 (thrombocytopenia), pyuria of renal origin.
Scarlet fever	Distributed equally among both genders. Most commonly affects children between five and fifteen years of age.	Occurs after streptococcal pharyngitis/tonsillitis	Pastia's sign (puncta and skin crease accentuation of the erythema), strawberry tongue, cervical lymphadenopathy may be present. Scarlet fever appears similar to Kawasaki's disease in some aspects, but lacks the eye signs or the swollen, red fingers and toes	Characteristic sandpaper-like rash which appears days after the illness begins (although the rash can appear before illness or up to 7 days later), rash may first appear on the neck, underarm, and groin	Leukocytosis with left shift and possibly eosinophilia a few weeks after convalescence. Anti-deoxyribonuclease B, antistreptolysin-O titers (antibodies to streptococcal extracellular products), antihyaluronidase, and antifibrinolysin may be positive.

Kawasaki disease must be differentiated from other causes of fever and rash in infants

Differential Diagnosis of Measles. Table adapted from CDC Pinkbook.

Disease	Agent	Typical Season	Typical Age	Prodrome	Fever	Duration of the rash (days)	Rash	Other Signs & Symptoms
Kawasaki disease	Unknown	Winter - Spring	< 5 years	3 days of abrupt fever	High; fever of 5 days is a diagnostic criteria	5 - 7	Erythematous, morbilliform, maculopapular or scarlatiniform, central distribution; erythematous, indurated palms and soles	Acute: dry, fissured and injected lips, strawberry tongue; irritability; cervical lymphadenopathy; conjunctival injection; peripheral edema; Subacute: finger-tip desquamation; Complications: arthritis, carditis
Measles	Paramyxovirus Measles virus	Winter - Spring	1 to 20 years	2-4 days of cough, conjunctivitis, and coryza	High	5 - 6	Erythematous, irregular size, maculopapular; starts on temples and behind ears; progresses down from face; fades to brownish	Koplik's spots: C blue-white papules (salt grains) on bright red mucosa opposite premolar teeth
Roseola Infantum (exanthem subitum)	Human herpes virus type 6	Any season	6 months to 2 years	None	High	1-2; it follows defervescence	Discrete erythematous macules, rarely involves face, begins as fever ends	Lymphadenopathy, irritability
Rubella	Togavirus	Spring	7 months to 29 years	0 - 4 days; mild malaise, fever; absent in children	Low grade	1 - 3	Discrete, rose-pink, diffuse, maculopapular; progresses downward from face, may change quickly	Arthralgia (usually in adults), tender posterior cervical and suboccipital lymphadenopathy, malaise, petechiae on soft palate
Scarlet Fever	ß-hemolytic streptococci	Winter	> 2 years	0 - 6 day, marked	Low to high	2 - 7	Scarlet "sunburn" with punctate papules "sandpaper", circumoral pallor, increased intensity in skin folds, blanches stars face/head, upper trunk and progresses downward	Sore throat, exudative tonsillitis, vomiting, abdominal pain, lmphadenopathy, white then red strawberry tongue
Erythema Infectiosum (Fifth Disease)	Human parvovirus type B19	Spring	5 - 10 years	None, usually in children, may occur in adults	None to low-grade	2 - 4	Starts as “slapped cheek”, maculopapular; progresses to reticular (lacy) pattern; can recur with environmental changes such as sunlight exposure	Arthralgia/arthritis in adults, adenopathy
Enterovirus	Echovirus Coxsackie virus	Summer - Fall	Mainly childhood	0 - 1 day fever and myalias	Low to high	1 - 5	Fine, pink, always affects face; variant is Boston exanthem (large ~ 1 cm, discrete maculopapules)	Sore throat, headache, malaise, no lymphadenopathy, gastroenteritis
Dengue Fever	Flavivirus Dengue virus types 1 - 4			None	High	1 - 5	Generalized maculopapular rash after defervescence; spares palms and soles	Headache, myalgia, abdominal pain, pharyngitis, vomiting
Drug induced rash	Many	Any	Any	Possible due to underlying illness	Possible	Varies	Typically diffuse but may be concentrated in diaper area, typically no progression, erythema multiform rash can progress over a few days	Possibly due to underlying illness or complications
Infectious Mononucleosis	Epstein-Barr Virus	None	10 - 30 years	2 - 5 days of malaise and fatigue	Low to high	2 - 7	Trunk and proximal extremities. Rash common if Ampicillin given	Pharyngitis, lymphadenopathy, splenomegaly, malaise
Pharyngoconjunctival Fever	Adenovirus types 2, 3, 4, 7, 7a	Winter - Spring	< 5 years		Low to high	3 - 5	Starts on face and spreads down to trunk and extremities	Sore throat, conjunctivitis, headache, anorexia

The following table is a list of differential diagnosis oral lesions presenting similar to measles:

Disease	Presentation	Risk Factors	Diagnosis	Affected Organ Systems	Important features	Picture
Coxsackie virus	Fever Sores in the mouth Rash with blisters Aches	Pregnancy immunodeficiency	History and Physical exam Throat swabs Swabs from the lesion Tzanck test	Oral cavity Skin	Symptomatic treatment	<figure-inline><figure-inline><figure-inline><figure-inline></figure-inline></figure-inline></figure-inline></figure-inline>
Chicken pox	Conjunctival symptoms Catarrhal symptoms Characteristic spots on the trunk appearing in two or three waves Itching	Pregnancy Premature infants born to susceptible mothers All infants born at less than 28 weeks gestation or who weigh ≤1000 grams Immunocompromised	History and physical exam PCR to detect VZV in skin lesions (vesicles, scabs, maculopapular lesions)	Oral cavity Skin	Sodium bicarbonate in baths or antihistamines for itching Paracetamol (acetaminophen) for fever Prednisolone is contraindicated	<figure-inline><figure-inline><figure-inline><figure-inline></figure-inline></figure-inline></figure-inline></figure-inline>
Measles	Fever Rash Cough Coryza (runny nose) Conjunctivitis (pink eye) Malaise Koplick spots in mouth	Unvaccinated individuals Crowded and/or unsanitary conditions Traveling to less developed and developing countries Immunocompromized Winter and spring seasons Born after 1956 and never fully vaccinated Health care workers	History and examination PCR for Measles-specific IgM antibody PCR for Measles RNA	Oral cavity Skin Respiratory tract Eyes Throat	Caused by Morbillivirus Primary site of infection is the respiratory epithelium of the nasopharynx Transmitted in respiratory secretions, via aerosol droplets containing virus particles	<figure-inline><figure-inline><figure-inline><figure-inline></figure-inline></figure-inline></figure-inline></figure-inline>
Herpangina	Sudden fever Sore throat and dysphagia- These can occur several hours(up to 24 hours), before the appearance of the enanthem. Vomiting Abdominal pain Myalgia Headache Pharyngeal lesions	Attendance at a kindergarten/child care center Contact with herpangina cases Residence in rural areas Overcrowding Poor hygiene Low socioeconomic status	Clincial diagnosis Pharyngeal viral and bacterial cultures can be taken to exclude HSV infection and streptococcal pharyngitis.	Skin Oral Cavity	Characteristic enanthem- Punctate macule which evolve over a period of 24 hours to 2-4mm erythematous papules which vesiculate, and then centrally ulcerate. The lesions are usually small in number, and evolve rapidly. The lesions are seen more commonly on the soft palate and uvula. The lesions can also be seen on the tonsils, posterior pharyngeal wall and the buccal mucosa.	Erythema, vesicles and ulcerating lesions in herpangina
Primary herpetic gingivoestomatitis	Pin-head vesicles rupture to form painful irregular ulcerations covered by yellow-grey membrane Severe pain Submandibular lymphadenopathy Halitosis It involves buccal mucosa, tongue, posterior pharynx, and gingival and palatal mucosa	Direct contact HIV infection	Tzanck test demonstrates multinucleated epithelial giant cells Viral culture is the gold standard for diagnosis Direct immunofluorescence	Oral cavity Mucous membranes	Ulcers are common on lips, gums, throat, front of tongue, inside of the cheeks and roof of the mouth Treatment is with antiviral agents such as Valacyclovir and Famciclovir	<figure-inline><figure-inline><figure-inline><figure-inline></figure-inline></figure-inline></figure-inline></figure-inline>

Koplik spots must be differentiated from other diseases causing oral lesions such as leukoplakia and herpes simplex virus infection.

Disease	Presentation	Risk Factors	Diagnosis	Affected Organ Systems	Important features	Picture
Diseases predominantly affecting the oral cavity
Oral Candidiasis	Dysphagia or odynophagia White patches on the mouth and tongue	Newborn babies Denture users Poorly controlled diabetes As a side effect of medication, most commonly having taken antibiotics. Inhaled corticosteroids for the treatment of lung conditions (e.g, asthma or COPD) may also result in oral candidiasis which may be reduced by regularly rinsing the mouth with water after taking the medication. People with poor nutrition, specifically vitamin A, iron and folate deficiencies. People with an immune deficiency (e.g. as a result of AIDS/HIV or chemotherapy treatment). Women undergoing hormonal changes, like pregnancy or those on birth control pills. Organ transplantation patients	Clinical diagnosis Confirmatory tests rarely needed	Localized candidiasis Oral and esophageal candidasis Candida vulvovaginitis Chronic mucocutaneous candidiasis Invasive candidasis Candidaemia Candida endocarditis Candida osteoarticular disease	Oral candidiaisis is a benign self limiting disease unless accompanied by immunosuppression.	Tongue infected with oral candidiasis - By James Heilman, MD - Own work, CC BY-SA 3.0, httpscommons.wikimedia.orgwindex.phpcurid=11717223.jpg
Herpes simplex oral lesions	Fever Sore throat Painful ulcers	Stress Recent URTI Female sex	Physical examination Viral culture Tzanck smear	Orofacial Infection Anogenital Infection Ocular Infection Herpes Encephalitis Neonatal Herpes Herpetic Whitlow Herpes Gladiatorum	The symptoms of primary HSV infection generally resolve within two weeks	Oral herpes simplex infection - By James Heilman, MD - Own work, CC BY-SA 3.0, httpscommons.wikimedia.orgwindex.phpcurid=19051042.jpg
Aphthous ulcers	Painful, red spot or bump that develops into an open ulcer	Being a female Between the ages of 10-40 Family history of aphthous ulcers	Physical examination Diagnosis of exclusion	Oral cavity	Self-limiting , Pain decreases in 7 to 10 days, with complete healing in 1 to 3 weeks	Apthous ulcer on the lower surface of the tongue - By Ebarruda - Own work, CC BY-SA 3.0, httpscommons.wikimedia.orgwindex.phpcurid=7903358
Squamous cell carcinoma	Non healing ulcer, nodule, indurated plaque or mass May involve skin, lips, inside the mouth, throat or esophagus	Chronic sun or UV exposure Fair skin Elderly age (>45 yrs) Male sex Smoking	Physical exam Biopsy	Oral Cavity Floor of mouth Lateral tongue Throat Esophagus	Malignant Can spread to TMJ Some times associated with leukoplakia	Squamous cell carcinoma - By Luca Pastore, Maria Luisa Fiorella, Raffaele Fiorella, Lorenzo Lo Muzio - http://www.plosmedicine.org/article/showImageLarge.action?uri=info%3Adoi%2F10.1371%2Fjournal.pmed.0050212.g001, CC BY 2.5, https://commons.wikimedia.org/w/index.php?curid=15252632
Leukoplakia	White leathery spots on the mucous membranes of the tongue and inside of the mouth Lateral borders of tongue	Atypical Tobacco use Chronic irritation Immunodeficiency Bloodroot (sanguinaria)	Physical exam Diagnosis of exclusion Biopsy	Vulvar lesions occur independent of oral lesions	Associated with HIV Persistant white spots Benign but can progress to carcinoma after almost 10 years Oral proliferative verrucous leukoplakia is an aggressive sub type with multiple lesions and higher conversion to warts or carcinoma	Leukoplakia - By Aitor III - Own work, Public Domain, https://commons.wikimedia.org/w/index.php?curid=9873087
Melanoma	A lesion with ABCD Asymmetry Border irregularity Color variation Diameter changes Bleeding from the lesion	UV radiations Genetic predisposition Old age Male gender Family or personal history of melanoma Multiple benign or atypical nevi	ABCD characteristics Bleeding or ulceration may show malignancy Serum LDH may be elevated in case of malignancy Biopsy	Can metastasize All UV radiation or sun exposed areas can be effected independently 1-2 to hundreds of granules	Neural crest cell derivative Development begins with disruption of nevus growth control Progression involves MAPK/ERK pathway N-RAS or BRAF oncogene also involved	Oral melanoma - By Emmanouil K Symvoulakis, Dionysios E Kyrmizakis, Emmanouil I Drivas, Anastassios V Koutsopoulos, Stylianos G Malandrakis, Charalambos E Skoulakis and John G Bizakis - Symvoulakis et al. Head & Face Medicine 2006 2:7 doi:10.1186/1746-160X-2-7 (Open Access), [1], CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=9839811
Fordyce spots	Rice-like granules or spots Small, painless, raised, pale, red or white 1 to 3 mm in diameter	Greasy skin types Some rheumatic disorders Hereditary nonpolyposis colorectal cancer Lower gingiva (gums) Vestibular mucosa	Physical exam Small keratin-filled pseudocysts May be seen on incidental mucosal biopsy Biopsy not done for them primarily	Oral cavity Vermilion border of the lips Oral mucosa of the upper lip Buccal mucosa in the commissural region often bilaterally Genitals	Benign neoplasms with sebaceous features Visible sebaceous glands No surrounding mucosal change Several adjacent glands may coalesce into a larger cauliflower-like cluster	Fordyce spots - Por Perene - Obra do próprio, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=19772899
Burning mouth syndrome	Burning or tingling on the lips, tongue, or entire mouth	Nutritional deficiencies Chronic anxiety or depression Diabetes type 2 Menopause Oral thrush or dry mouth, or damaged nerves transmitting taste Female gender Menopause	Presentation Physical exam	Oral cavity	Pain typically is low in the morning and builds up over the day Low dosages of benzodiazepines, tricyclic antidepressants or anticonvulsants may be effective
Torus palatinus	Bony growth on midline of the hard palate Nodular mass covered with normal mucosa	Genetic predisposition Autosomal dominant	Physical exam Types Flat tori Spindle tori Nodular tori Lobular tori	Hard palate	More common in Asian and Inuit populations Twice more common in females Repeated trauma can cause bleeding Surgery may be required in symptomatic	Torus palatinus - By Photo taken by dozenist, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=846591
Diseases involving oral cavity and other organ systems
Behcet's disease	Painful mouth sores Acne like skin lesions Headache, fever, poor balance, disorientation Abdominal pain, diarrhea or bleeding Uveitis Joint swelling and joint pain Genital sores wit pain and scaring Aneurysms	Over active immune system	Physical examination	Mouth Genitals GIT Eye Joints Skin Vascular system Brain	Outbreaks of exaggerated inflammation Affects smaller blood vessels	Behcet's disease - By Ahmet Altiner MD, Rajni Mandal MD - http://dermatology.cdlib.org/1611/articles/18_2009-10-20/2.jpg, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=17863021
Crohn's disease	Chronic, episodic diarrhea or constipation Abdominal pain Vomiting Weight loss or weight gain	Smoking Whites and European Jews Hormonal contraception Diets high in microparticles, sweet, fatty or refined foods Industrialized country	Typical history and symptoms Skip lesions on biopsy Anti-Saccharomyces cerevisiae antibodies (ASCA) Anti-neutrophil cytoplasmic antibodies (ANCA)	Eyes Joints Skin	May lead to Obstructions Abscesses Free perforation Hemorrhage
Agranulocytosis	Fever or chills Frequent infections Unusual redness, pain, or swelling around a wound Mouth ulcers Abdominal pain Burning sensation when urinating Sore throat	Medications Cytotoxic chemotherapy Hematologic malignancies Autoimmune disorders	Neutropenia <100 cells per micro litre Eosinopenia Basopenia	Oral cavity Skin GIT Urinary system Conjunctiva	Immunocompromization Types Drug-induced Malignant Autoimmune
Syphilis	Chancre Regional lymphadenopathy	Multiple sexual partners Illicit drug use Unprotected sex Men who have sex with men Residence in highly prevalent areas HIV infection Presence of other STIs Previous history of STIs Intravenous drug use	Darkfield microscopy Non treponemal tests like VDRL and RPR test) Treponemal testsFTA-ABS tests, (TP-PA) assay, enzyme immunoassays, and chemiluminescence immunoassays)	Oral cavity Penis Cervix Labia Anal canal Rectum CNS CVS	Primary syphilis Chancre Secondary syphilis Condylomata lata Latent syphilis Asymptomatic Tertiary syphilis Gummas Neurosyphilis	oral syphilis - By CDC/Susan Lindsley - http://phil.cdc.gov/phil_images/20021114/34/PHIL_2385_lores.jpg, Public Domain, https://commons.wikimedia.org/w/index.php?curid=2134349
Coxsackie virus	Fever Sores in the mouth Rash with blisters Aches	Pregnancy immunodeficiency	History and Physical exam Throat swabs Swabs from the lesion Tzanck test	Oral cavity Skin	Symptomatic treatment	Coxsackie virus stomatitis - Adapted from Dermatology Atlas.
Chicken pox	Conjunctival symptoms Catarrhal symptoms Characteristic spots on the trunk appearing in two or three waves Itching	Pregnancy Premature infants born to susceptible mothers All infants born at less than 28 weeks gestation or who weigh ≤1000 grams Immunocompromised	History and physical exam PCR to detect VZV in skin lesions (vesicles, scabs, maculopapular lesions)	Oral cavity Skin	Sodium bicarbonate in baths or antihistamines for itching Paracetamol (acetaminophen) for fever Prednisolone is contraindicated	Chickenpox - By James Heilman, MD - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=52872565
Measles	Fever Rash Cough Coryza (runny nose) Conjunctivitis (pink eye) Malaise Koplick spots in mouth	Unvaccinated individuals Crowded and/or unsanitary conditions Traveling to less developed and developing countries Immunocompromized Winter and spring seasons Born after 1956 and never fully vaccinated Health care workers	History and examination PCR for Measles-specific IgM antibody PCR for Measles RNA	Oral cavity Skin Respiratory tract Eyes Throat	Caused by Morbillivirus Primary site of infection is the respiratory epithelium of the nasopharynx Transmitted in respiratory secretions, via aerosol droplets containing virus particles	Koplick spots (Measles) - By CDC - http://phil.cdc.gov/PHIL_Images/20040908/4f54ee8f0e5f49f58aaa30c1bc6413ba/6111_lores.jpg, Public Domain, https://commons.wikimedia.org/w/index.php?curid=824483

Epidemiology and Demographics

KS occurs worldwide, with the highest incidence in Japan, and it most often affects boys and younger children. KS may have a winter-spring seasonality, and community-wide outbreaks have been reported occasionally. In the continental United States, population-based and hospitalization studies have estimated an incidence of KS ranging from 9 to 19 per 100,000 children younger than 5 years of age. Approximately 4248 hospitalizations for KS, of which 3277 (77%) were for children under 5 years of age, were estimated among children younger than 18 years of age in the United States in the year 2000.

CDC uses hospital discharge data, a passive KS surveillance system, and special studies to describe the incidence and epidemiology of KS in the United States. The KS surveillance system has been maintained by CDC since 1976 and is based on voluntary reporting of KS cases by health care providers and local and state health authorities. A standardized case report form is used to collect information on patients.

For epidemiologic surveillance, CDC defines a case of KS as illness in a patient with fever of 5 or more days duration (or fever until the date of administration of intravenous immunoglobulin if it is given before the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:

• Rash
• Cervical lymphadenopathy (at least 1.5 cm in diameter)
• Bilateral conjuctival injection
• Oral mucosal changes
• Peripheral extremity changes.

Patients whose illness does not meet the above KS case definition but who have fever and coronary artery abnormalities are classified as having atypical or incomplete KS.

Incidence

By far, the highest incidence of Kawasaki disease occurs in Japan (175 per 100,000), though its incidence in the United States is increasing. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. Additional risk factors in the United States include Asian race and male sex.

Natural History, Complications, and Prognosis

Prognosis

The cardiac complications are, by far, the most important aspect of the disease. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction (heart attack) even in young children. Overall, about 10–18% of children with Kawasaki disease develop coronary artery aneurysms, with much higher prevalence among patients who are not treated early in the course of illness. Kawasaki disease is the most common cause of acquired heart disease among children in the United States.

With early treatment, rapid recovery from the acute symptoms can be expected and the risk of coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery involvement is much greater. Overall, about 2% of patients die from complications of coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every 1–2 years to screen for progression of cardiac involvement.

It is also not uncommon that a relapse of symptoms may occur soon after initial treatment with IVIG. This usually requires re-hospitalization and retreatment. Treatment with IVIG can cause allergic and non-allergic acute reactions, aseptic meningitis, fluid overload and, rarely, other serious reactions. Aspirin may increase the risk of bleeding from other causes and may be associated with Reye's syndrome. Overall, life-threatening complications resulting from therapy for Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment.

Diagnosis

Diagnostic Criteria

Kawasaki disease is diagnosed clinically (by medical signs and symptoms), and there exists no specific laboratory test that can tell if someone has it. It is normally difficult to establish the diagnosis, especially early in the course of illness, and frequently children are not diagnosed until they have seen their doctor several times, or visited a number of different health care providers. Many other serious illnesses can cause similar symptoms, and must be considered in the differential diagnosis, including scarlet fever, toxic shock syndrome, and juvenile idiopathic arthritis.

Classically, five days of fever plus four of five diagnostic criteria must be met in order to establish the diagnosis.

The criteria are:

(1) Mucositis: erythema of the palatine mucosa, fissure erythematous lips, "strawberry tongue"

(2) Rash: Polymorphus, usually urticarial erythematous rash mainly in external extremities. The rash can spread to trunk

(3) Extremities changes: Edema of hand and feet, erythema of palms & soles, desquamation of fingertips

(4) Bilateral non-exudative conjuctival erythema

(5) Cervical lymphadenopathy for at least 15 milimeters

Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting.

History and Symptoms

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of acetaminophen or ibuprofen. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes, red mucous membranes in the mouth, red cracked lips, a "strawberry tongue", iritis, keratic precipitates (detectable by an ophthalmologist but usually too small to be seen by the unaided eye), and swollen lymph nodes. Skin rashes occur early in the disease, and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short.

• High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F) that normally lasts for more than a week if left untreated.
• Red eyes (conjunctivitis) without pus or drainage, also known as "conjunctival injection"
• Bright red, chapped, or cracked lips
• Red mucous membranes in the mouth
• Strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue
• Red palms of the hands and the soles of the feet
• Swollen hands and feet
• Rash which may take many forms, but not vesicular (blister-like), on the trunk
• Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area
• Joint pain (arthralgia) and swelling, frequently symmetrical
• Irritability
• Tachycardia (rapid heart beat)
• Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails

AHA Scientific Statement on Kawasaki Disease

Recommendations for Cardiovascular Assessment for Diagnosis and Monitoring During the Acute Illness

Class I

"1. Echocardiography should be performed when the diagnosis of KD is considered, but unavailability or technical limitations should not delay treatment.(Level of Evidence: B) "

"2. Coronary arteries should be imaged, and quantitative assessment of luminal dimensions, normalized as Z scores adjusted for body surface, should be performed.(Level of Evidence: B) "

"3. For uncomplicated patients, echocardiog- raphy should be repeated both within 1 to 2 weeks and 4 to 6 weeks after treatment.(Level of Evidence: B) "

"4. For patients with important and evolving coronary artery abnormalities (Z score >2.5) detected during the acute illness, more fre- quent echocardiography (at least twice per week) should be performed until luminal dimensions have stopped progressing to determine the risk for and presence of thrombosis.(Level of Evidence: B) "

Class IIa

"1. To detect coronary artery thrombosis, it may be reasonable to perform echocardiography for patients with expanding large or giant aneurysms twice per week while dimensions are expanding rapidly and at least once weekly in the first 45 days of illness, and then monthly until the third month after illness onset, because the failure to escalate thromboprophylaxis in time with the rapid expansion of aneurysms is a primary cause of morbidity and mortality . (Level of Evidence: C) "

Physical Examination

A physical examination will demonstrate many of the features listed above.

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Blood tests

• Complete blood count (CBC) may reveal normocytic anemia and eventually thrombocytosis
• Erythrocyte sedimentation rate (ESR) will be elevated
• C-reactive protein (CRP) will be elevated
• Liver function tests may show evidence of hepatic inflammation and low serum albumin

Other Diagnostic Studies

Other tests (may or may not be performed)

• Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia due to myocarditis
• Echocardiogram may show subtle coronary artery changes or, later, true aneurysms.
• Ultrasound or computerized tomography may show hydrops (enlargement) of the gallbladder
• Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria) without evidence of bacterial growth
• Lumbar puncture may show evidence of aseptic meningitis
• Angiography was historically used to detect coronary artery aneurysms and remains the gold standard for their detection, but is rarely used today unless coronary artery aneurysms have already been detected by echocardiography.

Treatment

Medical Therapy

Intravenous Immunoglobulin (IVIG) and aspirin are indicated in the treatment of Kawasaki Disease. It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the coronary arteries. Except for Kawasaki disease and a couple of other indications, aspirin is otherwise normally not recommended for children due to its association with Reye's syndrome. Children with Kawasaki disease should be hospitalized.

• 1. Initial treatment

• Preferred regimen: IVIG 2 g/kg single infusion within the first 7-10 days of illness AND Aspirin 80-100 mg/kg/day qid , reduce the aspirin dose after the child has been afebrile for 48 to 72 hours, then begin low-dose aspirin (3 to 5 mg/kg/day) and maintain it until the patient shows no evidence of coronary changes by 6 to 8 weeks after the onset of illness
• Note (1): Other clinicians continue highdose aspirin until day 14 of illness and 48 to 72 hours after fever cessation
• Note (2): For children who develop coronary abnormalities, aspirin may be continued indefinitely

• 2. Treatment of Patients Who Failed to Respond to Initial Therapy (persistent or recrudescent fever ≥ 36 hours after completion of the initial IVIG infusion)

• Preferred regimen: IVIG 2 g/kg q24h for 1-3 days OR Methylprednisolone 30 mg/kg IV for 2-3 hours q24h for 1-3 days

AHA Scientific Statement on Kawasaki Disease

Recommendations for Initial Treatment With Intravenous Immunoglobulin (IVIG) and Asetil Salisilat Acid (ASA)

Class I

"1. Patients with complete KD criteria and those who meet the algorithm criteria for incomplete KD should be treated with high-dose IVIG (2 g/kg given as a single intravenous infusion) within 10 days of illness onset but as soon as possible after diagnosis.(Level of Evidence: A) "

Class IIa

"1. It is reasonable to administer IVIG to children presenting after the 10th day of illness (ie, in whom the diagnosis was missed earlier) if they have either persistent fever without other explanation or coronary artery abnormalities together with ongoing systemic inflammation, as manifested by elevation of ESR or CRP (CRP >3.0 mg/dL). (Level of Evidence: B) "

"2. Administrationofmoderate-(30–50mg·kg−1·d−1) to high-dose (80–100 mg·kg−¹·d−¹) ASA is reasonable until the patient is afebrile, although there is no evidence that it reduces coronary artery aneurysms. (Level of Evidence: C) "

Class III

"1. IVIG generally should not be administered to patients beyond the tenth day of illness in the absence of fever, significant elevation of inflammatory markers, or coronary artery abnormalities . (Level of Evidence: C) "

"2. The ESR is accelerated by IVIG therapy and therefore should not be used to assess response to IVIG therapy. A persistently high ESR alone should not be interpreted as a sign of IVIG resistance. (Level of Evidence: C) "

Recommendations for Adjunctive Therapies for Primary Treatment

Class IIb

"1. Administration of a longer course of corticosteroids (eg, tapering over 2–3 weeks), together with IVIG 2 g/kg and ASA, may be considered for treatment of high-risk patients with acute KD, when such high risk can be identified in patients before initiation of treatment. (Level of Evidence: B) "

Class III

"1. Single-dose pulse methylprednisolone should not be administered with IVIG as routine primary therapy for patients with Kawasaki Disease. (Level of Evidence: B) "

Recommendations for Additional Therapy in the IVIG-Resistant Patient

Class IIa

"1. It is reasonable to administer a second dose of IVIG (2 g/kg) to patients with persistent or recrudescent fever at least 36 hours after the end of the first IVIG infusion. (Level of Evidence: B) "

Class IIb

"1. Administration of high-dose pulse steroids (usually methylprednisolone 20–30 mg/kg intravenously for 3 days, with or without a subsequent course and taper of oral prednisone) may be considered as an alternative to a second infusion of IVIG or for retreatment of patients with KD who have had recurrent or recrudescent fever after additional IVIG. (Level of Evidence: B) "

"2. Administration of a longer (eg, 2–3 weeks) tapering course of prednisolone or prednisone, together with IVIG 2 g/kg and ASA, may be considered in the retreatment of patients with KD who have had recurrent or recrudescent fever after initial IVIG treatment. (Level of Evidence: B) "

"3. Administration of infliximab (5 mg/kg) may be considered as an alternative to a second infusion of IVIG or corticosteroids for IVIG-resistant patients. (Level of Evidence: C) "

"4. Administration of cyclosporine may be considered in patients with refractory KD in whom a second IVIG infusion, infliximab, or a course of steroids has failed. (Level of Evidence: C) "

"5. Administration of immunomodulatory monoclonal antibody therapy (except TNF-α block- ers), cytotoxic agents, or (rarely) plasma exchange may be considered in highly refractory patients who have failed to respond to a second infusion of IVIG, an extended course of steroids, or infliximab. (Level of Evidence: C) "

Recommendations for Treatment of Coronary Artery Thrombosis

Class I

"1. Coronary artery thrombosis with actual or impending occlusion of the arterial lumen should be treated with thrombolytic therapy or, in patients of sufficient size, by mechanical restoration of coronary artery blood flow at cardiac catheterization.(Level of Evidence: C) "

"2. Thrombolytic agents should be administered together with low-dose ASA and low-dose heparin, with careful monitoring for bleeding.(Level of Evidence: C) "

Class IIb

"1. Treatment of coronary artery thrombosis with substantial thrombus burden and high risk of occlusion with a combination of reduced-dose thrombolytic therapy and abciximab may be considered. (Level of Evidence: C) "

Risk-Stratified Recommendations for Long-Term Evaluation and Management

No Involvement (Z Score Always <2)

Class IIa

"1. It is reasonable to use echocardiographic coronary artery luminal dimensions converted to BSA-adjusted Z scores to determine risk stratification. (Level of Evidence: B) ". (Level of Evidence: C) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable that no additional cardiology assessment be performed. (Level of Evidence: B) ". (Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may be provided by the primary care provider. (Level of Evidence: B) ". (Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, fast- ing lipid profile, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking at least once and ideally at least 1 year from the episode of acute KD; this may be performed by the primary care provider. (Level of Evidence: B) ". (Level of Evidence: C) "

Medical therapy (β-blockers, angiotensin-converting enzyme inhibitor [ACEI], statin)

Class III

"1. No additional medical therapy should be given. (Level of Evidence: C) "

Thromboprophylaxis

Class IIa

"1. It is reasonable to give low-dose ASA for up to 4 to 6 weeks after the episode of acute KD, which should be discontinued thereafter. (Level of Evidence: C) ". (Level of Evidence: C) "

Physical activity

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit with no restrictions or precautions at any time. (Level of Evidence: B) ". (Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to provide age-appropriate counseling regarding contraception and pregnancy without modification. (Level of Evidence: B) ". (Level of Evidence: C) "

Dilation Only (Z Score ≥2 but <2.5, or a Decrease in Z Score During Follow-up ≥1)

Class IIa

"1. If luminal dimensions have returned to normal by 4 to 6 weeks after KD onset, it is reasonable to discharge the patient from cardiology care, although ongoing follow-up to 12 months may be considered. (Level of Evidence: B) ". (Level of Evidence: C) "

"2. If dilation remains present at 4 to 6 weeks after KD onset, then it is reasonable to continue follow-up to 12 months. If the luminal dimensions return to normal before then, it is reasonable to discharge the patient from ongoing cardiology care. (Level of Evidence: B) ". (Level of Evidence: C) "

"3. Resolution is expected within 1 year. If dilation persists at 1 year, consider whether this represents a dominant branch. If this is a probable explanation, then it is reasonable to discharge the patient from ongoing cardiology care, although ongoing follow-up every 2 to 5 years may be considered. Patients and families should be advised to remember that having had KD is part of the patient’s permanent medical history. (Level of Evidence: C) ". (Level of Evidence: C) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable that no additional cardiology assessment be performed. (Level of Evidence: B) ". (Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may be provided by the primary care provider. (Level of Evidence: C) ". (Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, fasting lipid profile, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking at least once and ideally at least 1 year from the episode of acute KD; this may be performed by the primary care provider. (Level of Evidence: C) ". (Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class III

"1. No additional medical therapy should be given. (Level of Evidence: C) "

Thromboprophylaxis

Class IIa

"1. It is reasonable to give low-dose ASA until 4 to 6 weeks after the acute episode, which should be discontinued thereafter. (Level of Evidence: C) ". (Level of Evidence: C) "

Physical activity

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit with no restrictions or precautions at any time. (Level of Evidence: B) "

Reproductive counseling

Class IIa

"1. It is reasonable to provide age-appropriate counseling regarding contraception and pregnancy without modification. (Level of Evidence: B) "

Regression to Normal Z Score or Dilation Only

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIa

"1. It is reasonable to assess every 1 to 3 years. It is reasonable not to perform echocardiogra- phy unless there is evidence for inducible myocardial ischemia or the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction. (Level of Evidence: B) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myocardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every 3 to 5 years or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction. (Level of Evidence: B) "

Class IIb

"1. Further imaging with angiography (CT, MRI, invasive) may be considered only if there is evidence for inducible myocardial ischemia or ventricular dysfunction. (Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, fasting lipid profile, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking every 2 years; this may be performed by the primary care provider. It is reasonable to obtain a follow- up fasting lipid profile. (Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

Class III

"1. Empirical treatment with β-blockers is not indicated.(Level of Evidence: C) "

Thromboprophylaxis

Class IIb

"1. Ongoing treatment with low-dose ASA may be considered, although it is reasonable to discontinue.(Level of Evidence: C) "

Class IIa

"1. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intol- erant or resistant to ASA.(Level of Evidence: C) "

Physical activity

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit with no restrictions or precautions at any time .(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to provide age-appropriate counseling regarding contraception and pregnancy without modification.(Level of Evidence: B) "

Medium Aneurysms (Z Score ≥5 to <10, With an Absolute Luminal Dimension <8 mm)

Current or Persistent Medium Aneurysms

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit with no restrictions or precautions at any time .(Level of Evidence: B) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myo- cardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every 1 to 3 years or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: B) "

Class IIb

"1. Further imaging with angiography (CT, MRI, invasive) may be considered for periodic sur- veillance every 2 to 5 years.(Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, fasting lipid profile, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking at least once and ideally at least 1 year from the episode of acute KD; this may be performed by the primary care provider. It is reasonable to obtain a follow-up fasting lipid profile.(Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

Class III

"1. Empirical treatment with β-blockers is not indicated.(Level of Evidence: C) "

Thromboprophylaxis

Class I

"1. Patients should be treated with low-dose ASA.(Level of Evidence: C) "

Class IIa

"1. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intoler- ant or resistant to ASA.(Level of Evidence: C) "

Class IIb

"1. Additional patient and coronary artery characteristics may be considered in decision making regarding intensification of thromboprophylaxis.(Level of Evidence: C) "

"2. Dual-antiplatelet therapy with an additional antiplatelet agent (eg, a thienopyridine such as clopidogrel) may be considered.(Level of Evidence: C) "

Class III

"1. Use of anticoagulation (warfarin, LMWH) is not indicated.(Level of Evidence: C) "

Physical activity

Class I

"1. For patients taking dual-antiplatelet therapy, activities involving a risk of bodily contact, trauma, or injury should be restricted or modified.(Level of Evidence: B) "

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit without restrictions or precautions. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to discourage use of oral contraceptive drugs that increase thrombosis risk, to recommend that pregnancy be supervised by a multidisciplinary team including a cardiologist, and to alter thromboprophylaxis management during pregnancy and delivery.(Level of Evidence: B) "

Regression to Small Aneurysms

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIa

"1. Ongoing follow-up assessment every year is reasonable.(Level of Evidence: B) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myocardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every 2 to 3 years or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: B) "

Class IIb

"1. Further imaging with angiography (CT, MRI, invasive) may be considered for periodic surveillance every 3 to 5 years.(Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, fasting lipid profile, body mass index (and plot), waist circumference, dietary and activ- ity assessment, and smoking every year; this may be performed by the primary care provider. It is reasonable to obtain a follow- up fasting lipid profile.(Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

Class III

"1. Empirical treatment with β-blockers is not indicated.(Level of Evidence: C) "

Thromboprophylaxis

Class I

"1. Patients should be treated with low-dose ASA.(Level of Evidence: C) "

Class IIa

"1. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intolerant or resistant to ASA.(Level of Evidence: C) "

Class IIb

"1. Dual-antiplatelet therapy with an additional antiplatelet agent (eg, a thienopyridine such as clopidogrel) may be considered.(Level of Evidence: C) "

"2. Additional patient and coronary artery characteristics may be considered in decision making regarding intensification or discontinuation of thromboprophylaxis.(Level of Evidence: C) "

Class III

"1. Use of anticoagulation is not indicated.(Level of Evidence: C) "

Physical activity

Class I

"1. For patients taking dual-antiplatelet therapy, activities involving a risk of bodily contact, trauma, or injury should be restricted or modified.(Level of Evidence: B) "

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit without restrictions or precautions. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to discourage use of oral contraceptive drugs that increase thrombo- sis risk, to recommend that pregnancy be supervised by a multidisciplinary team includ- ing a cardiologist, and to alter thromboprophylaxis management during pregnancy and delivery.(Level of Evidence: B) "

Regression to Normal Z Score or Dilation Only

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIb

"1. It is reasonable to discourage use of oral contraceptive drugs that increase thrombo- sis risk, to recommend that pregnancy be supervised by a multidisciplinary team includ- ing a cardiologist, and to alter thromboprophylaxis management during pregnancy and delivery.(Level of Evidence: B) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myocardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every 2 to 4 years or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: B) "

"2. It is reasonable to perform no further imaging with angiography (CT, MRI, invasive) in the absence of evidence of inducible myocardial ischemia. (Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, fasting lipid profile, body mass index (and plot), waist circumference, dietary and activ- ity assessment, and smoking every 2 years; this may be performed by the primary care provider. It is reasonable to obtain a follow- up fasting lipid profile.(Level of Evidence: C) "

Medical therapy (β-blockers, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

Class III

"1. Empirical treatment with β-blockers is not indicated.(Level of Evidence: C) "

Thromboprophylaxis

Class IIa

"1. It is reasonable to continue treatment with low-dose ASA.(Level of Evidence: C) "

"2. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intolerant or resistant to ASA.(Level of Evidence: C) "

Class IIb

"1. Use of an additional antiplatelet agent (eg, a thienopyridine such as clopidogrel) is not recommended except in the presence of inducible myocardial ischemia.(Level of Evidence: C) "

"2. Additional patient and coronary artery characteristics may be considered in decision making regarding intensification or discontinuation of thromboprophylaxis.(Level of Evidence: C) "

Class III

"1. Use of anticoagulation (warfarin/LMWH) is not indicated.(Level of Evidence: C) "

Physical activity

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit without restrictions or precautions. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to provide age-appropriate counseling regarding contraception and pregnancy without modification.(Level of Evidence: B) "

Large and Giant Aneurysms (Z Score ≥10 or Absolute Dimension ≥8 mm)

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIa

"1. It is reasonable to assess patients at 1, 2, 3, 6, 9, and 12 months after the episode of acute KD in the first year and every 3 to 6 months thereafter.(Level of Evidence: C) "

Type and frequency of additional cardiology assess- ment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myocardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every 6 to 12 months or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: B) "

Class IIb

"1. Further imaging with angiography (CT, MRI, invasive) may be considered for diagnostic and prognostic purposes during the first year and may be considered for periodic surveillance every 1 to 5 years thereafter.(Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

Class IIa

"1. It is reasonable to assess blood pressure, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking every 6 to 12 months; this may be performed by the primary care provider. It is reasonable to obtain a fasting lipid profile during follow- up.(Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

"2. Empirical treatment with β-blockers may be considered .(Level of Evidence: C) "

Thromboprophylaxis

Class I

"1. Patients should be treated with low-dose ASA.(Level of Evidence: C) "

Class IIa

"1. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intolerant or resistant to ASA.(Level of Evidence: C) "

"2. Use of warfarin to achieve a target international normalized ratio of 2 to 3 is reasonable.(Level of Evidence: B) "

"3. Use of LMWH to achieve target anti-factor Xa levels of 0.5 to 1.0 U/mL is reasonable as an alternative to warfarin.(Level of Evidence: C) "

Class IIb

"1. Use of an additional antiplatelet agent (eg, a thienopyridine such as clopidogrel) may be considered together with ASA and warfarin/ LMWH (triple therapy) for thromboprophylaxis in the setting of very extensive or distal coronary artery aneurysms, or if there is a history of coronary artery thrombosis.(Level of Evidence: C) "

"2. Additional patient and coronary artery characteristics (Table 9) may be considered in decision making regarding adjustments to strategy for thromboprophylaxis.(Level of Evidence: C) "

Physical activity

Class I

"1. Activities involving a risk of bodily contact, trauma, or injury should be restricted or modified if the patient is on dual-antiplatelet or anticoagulation therapy.(Level of Evidence: B) "

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit without restrictions or precautions. Participation in competitive sports or high intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to discourage use of oral contraceptive drugs that increase thrombosis risk, to recommend that pregnancy be super- vised by a multidisciplinary team including a cardiologist, and to alter thromboprophylaxis management during pregnancy and delivery.(Level of Evidence: B) "

Regression to Medium Aneurysms

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIa

"1. It is reasonable to assess the patient every 6 to 12 months.(Level of Evidence: C) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myocardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every year or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: B) "

Class IIb

"1. Further imaging with angiography (CT, MRI, invasive) may be considered for periodic surveillance every 2 to 5 years.(Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking every year; this may be performed by the primary care provider. It is reason- able to obtain a follow-up fasting lipid profile.(Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

"2. Empirical treatment with β-blockers may be considered.(Level of Evidence: C) "

Thromboprophylaxis

Class I

"1. Patients should be treated with low-dose ASA.(Level of Evidence: C) "

Class IIa

"1. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intolerant or resistant to ASA.(Level of Evidence: C) "

"2. Discontinuation of anticoagulation (warfarin/ LMWH) and substitution with an additional antiplatelet agent (eg, a thienopyridine such as clopidogrel) is reasonable.(Level of Evidence: C) "

Class IIb

"1. Additional patient and coronary artery characteristics may be considered in decision making regarding adjustments to strategy for thromboprophylaxis.(Level of Evidence: C) "

Class III

"1. Use of anticoagulation (warfarin, LMWH) is not indicated.(Level of Evidence: C) "

Physical activity

Class I

"1. Activities involving a risk of bodily contact, trauma, or injury should be restricted or modified for patients on dual-antiplatelet or anticoagulation therapy.(Level of Evidence: B) "

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit without restrictions or precautions. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to discourage use of oral contraceptive drugs that increase thrombosis risk, to recommend that pregnancy be super- vised by a multidisciplinary team including a cardiologist, and to alter thromboprophylaxis management during pregnancy and delivery.(Level of Evidence: B) "

Regression to Small Aneurysms

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIa

"1. It is reasonable to assess the patient every 6 to 12 months.(Level of Evidence: C) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myocardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every 1 to 2 years or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: B) "

Class IIb

"1. Further imaging with angiography (CT, MRI, invasive) may be considered for periodic surveillance every 2 to 5 years.(Level of Evidence: C) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking every year; this may be performed by the primary care provider. It is reason- able to obtain a follow-up fasting lipid profile.(Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

"2. Empirical treatment with β-blockers may be considered.(Level of Evidence: C) "

"3. Discontinuation of additional medical therapy may be considered.(Level of Evidence: C) "

Thromboprophylaxis

Class I

"1. Patients should be treated with low-dose ASA.(Level of Evidence: C) "

Class IIa

"1. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intolerant or resistant to ASA.(Level of Evidence: C) "

Class IIb

"1. Additional patient and coronary artery char- acteristics may be considered in decision making regarding adjustments to strategy for thromboprophylaxis.(Level of Evidence: C) "

Class III

"1. Anticoagulation or dual-antiplatelet therapy is not indicated.(Level of Evidence: C) "

Physical activity

Class I

"1. For patients on anticoagulation or dual-antiplatelet therapy, activities involving a risk of bodily contact, trauma, or injury should be restricted or modified.(Level of Evidence: B) "

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit without restrictions or precautions. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to provide age-appropriate counseling regarding contraception. It is reasonable to recommend that pregnancy be supervised by a multidisciplinary team including a cardiologist and to alter thromboprophylaxis management during pregnancy and delivery.(Level of Evidence: B) "

Regression to Normal Z Score or Dilation Only

Frequency of cardiology assessment (to include history and physical examination, echocardiography, electrocardiography)

Class IIa

"1. It is reasonable to assess the patient every 1 to 2 years. Not performing routine 2D echocardiography may be considered unless there is evidence for inducible myocardial ischemia or the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: C) "

Type and frequency of additional cardiology assessment (other cardiology testing)

Class IIa

"1. It is reasonable to assess for inducible myocardial ischemia (stress echocardiography, stress with MRI, stress NM perfusion imaging, PET) every 2 to 5 years or if the patient has symptoms suggestive of ischemia or signs suggestive of ventricular dysfunction.(Level of Evidence: B) "

Cardiovascular risk factor assessment and management

Class IIa

"1. It is reasonable to provide general counseling regarding healthy lifestyle and activity promotion at every visit; this may additionally be provided by the primary care provider.(Level of Evidence: C) "

"2. It is reasonable to assess blood pressure, body mass index (and plot), waist circumference, dietary and activity assessment, and smoking every 2 years; this may be performed by the primary care provider. It is reasonable to obtain a follow-up fasting lipid profile as per the Expert Panel guidelines.(Level of Evidence: C) "

Medical therapy (β-blockers, ACEI, statin)

Class IIb

"1. Empirical statin therapy for non–lipid-lowering (pleiotropic) effects may be considered.(Level of Evidence: C) "

Class III

"1. Empirical treatment with β-blockers is not indicated.(Level of Evidence: C) "

Thromboprophylaxis

Class IIa

"1. It is reasonable to continue treatment with low-dose ASA.(Level of Evidence: C) "

"2. Use of an alternative antiplatelet agent (eg, a thienopyridine such as clopidogrel) instead of ASA is reasonable if the patient is intol- erant or resistant to ASA.(Level of Evidence: C) "

Class IIb

"1. Additional patient and coronary artery characteristics may be considered in decision making regarding intensification or discontinuation of thromboprophylaxis.(Level of Evidence: C) "

Class III

"1. Use of anticoagulation (warfarin/LMWH) or dual-antiplatelet therapy is not indicated.(Level of Evidence: C) "

Physical activity

Class I

"1. For patients on anticoagulation or dual-anti- platelet therapy, activities involving a risk of bodily contact, trauma, or injury should be restricted or modified.(Level of Evidence: B) "

Class IIa

"1. It is reasonable to provide physical activity counseling at every visit without restrictions or precautions. Participation in competitive sports or high-intensity activities should be guided by results from testing for inducible myocardial ischemia or exercise-induced arrhythmias.(Level of Evidence: C) "

Reproductive counselling

Class IIa

"1. It is reasonable to provide age-appropriate counseling regarding contraception. It is reasonable to recommend that pregnancy be supervised by a multidisciplinary team including a cardiologist and to alter thrombo- prophylaxis management during pregnancy and delivery.(Level of Evidence: B) "

Recommendations for Testing for Inducible Ischemia

Class IIa

"1. It is reasonable to use stress echocardiography or CMRI, NM MPI, or PET for assessment of inducible myocardial ischemia. Note: The general principle is to minimize risk to the patient, particularly cumulative radiation dose, and this should guide selection of testing modality based on patient and institutional characteristics(Level of Evidence: B) "

Class III

"1. Exercise treadmill electrocardiographic testing alone should not be used for assessment for inducible myocardial ischemia.(Level of Evidence: C) "

Recommendation for Assessment of Patients With Inducible Myocardial Ischemia

Class I

"1. Patients with evidence of inducible myocardial ischemia on testing should undergo invasive coronary angiography.(Level of Evidence: B) "

Recommendations for Indications for Mechanical Revascularization

Class I

"1. Adult patients with remote history of KD presenting with STEMI should be referred emergently for coronary angiography for determination of best means of flow restoration in the culprit artery.(Level of Evidence: C) "

"2. Revascularization should be performed in KD patients with stable angina and high-risk coronary anatomy including left main CAD, multi- vessel coronary disease with reduction in LV function, multivessel coronary disease with diabetes mellitus, or high-risk noninvasive ischemia testing.(Level of Evidence: C) "

"3. Revascularization should be performed for patients with non–ST-segment elevation and coronary anatomy amenable to revascularization on coronary angiography.(Level of Evidence: C) "

Class IIa

"1. Revascularization for patients with stable angina and symptoms refractory to maximal medical therapy is reasonable. (Level of Evidence: C) "

Class IIb

"1. Revascularization for KD patients with silent ischemia and ischemia involving >10% of LV mass may be considered. (Level of Evidence: C) "

Class III

"1. Revascularization should be avoided in KD patients in the acute/subacute phase of the illness with STEMI attributable to acute thrombotic occlusion of an aneurysm.(Level of Evidence: C) "

Recommendations for Modes of Revascularization

Class I

"1. CABG is preferred to PCI in KD patients with left main CAD, multivessel CAD with reduced LV function, multivessel CAD with lesions not amenable to PCI, and multivessel CAD in diabetic patients.(Level of Evidence: B) "

"2. CABG is preferred to PCI in older children and adults with KD and multivessel involvement .(Level of Evidence: C) "

"3. CABG should be performed with bilateral internal thoracic arterial grafts where possible.(Level of Evidence: B) "

"4. PCI is preferred in patients with single-vessel or focal multivessel disease amenable to PCI.(Level of Evidence: C) "

"5. RA and stents should be used in PCI of calcified lesions.(Level of Evidence: C) "

Class IIa

"1. The use of multivessel PCI is reasonable for KD patients with focal lesions amenable to PCI. (Level of Evidence: C) "

"2. The use of DESs during PCI is reasonable for KD patients who do not require long- term anticoagulation. (Level of Evidence: C) "

"3. The use of IVUS is reasonably indicated during PCI in KD patients to ensure adequate stent sizing and deployment. (Level of Evidence: C) "

Class IIb

"1. Multivessel PCI may be considered for patients who are acceptable CABG candidates but prefer to avoid CABG, provided the risks and benefits of both approaches are discussed with and understood by the patient. (Level of Evidence: C) "

"2. The use of DESs during PCI may be considered for KD patients who require anti- coagulation, provided the bleeding risk of the patient is acceptable. (Level of Evidence: C) "

Class III

"1. Stand-alone balloon angioplasty should not be used for PCI in KD patients with coronary obstructions.(Level of Evidence: C) "

Recommendation for Cardiac Transplantation

Class IIa

"1. The use of multivessel PCI is reasonable for KD patients with focal lesions amenable to PCI. (Level of Evidence: C) "

Prevention

AHA Scientific Statement on Kawasaki Disease

Recommendations for Prevention of Thrombosis During the Acute Illness

Class I

"1. Low-dose ASA (3–5 mg·kg−¹·d−¹) should be administered to patients without evidence of coronary artery changes until 4 to 6 weeks after onset of illness.(Level of Evidence: C) "

Class IIa

"1. For patients with rapidly expanding coronary artery aneurysms or a maximum Z score of ≥10, systemic anticoagulation with LMWH or warfarin (international normalized ratio target 2.0–3.0) in addition to low dose ASA is reasonable. (Level of Evidence: B) "

Class IIb

"1. For patients at increased risk of thrombosis, for example, with large or giant aneurysms (≥8 mm or Z score ≥10) and a recent history of coronary artery thrombosis, “triple therapy” with ASA, a second antiplatelet agent, and anticoagulation with warfarin or LMWH may be considered. (Level of Evidence: C) "

Class III

"1. Ibuprofen and other non steroidal anti-inflammatory drugs with known or potential involvement of cyclooxygenase pathway may be harmful in patients taking ASA for its antiplatelet effects. (Level of Evidence: B) "

Recommendations for Risk Stratification of Coronary Artery Abnormalities

Class IIa

"1. It is reasonable to use echocardiographic coronary artery luminal dimensions converted to BSA-adjusted Z scores to determine risk stratification. (Level of Evidence: B) "

"2. It is reasonable to incorporate both maximal and current coronary artery involvement in risk stratification. (Level of Evidence: C) "

"3. It is reasonable to incorporate the presence of additional features other than coronary artery luminal dimensions into decisions regarding risk stratification. (Level of Evidence: C) "

External links

• Kawasaki Disease Foundation
• Kawasaki Disease Forum
• Kawasaki Disease Canada
• Kawasaki Disease Research Program
• Kawasaki Disease information from Seattle Children's Hospital Heart Center
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