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|peptic ulcer disease
|Peptic ulcer disease
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* Epigastric pain
* Epigastric pain
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| rowspan="6" |Musculoskeletal
| rowspan="6" |Musculoskeletal
|arthritis
|Arthritis
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* arthralgias
* arthralgias
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|avascular necrosis
|Avascular necrosis
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* Asymptomatic
* Asymptomatic
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|bone fragility fractures
|Bone fragility fractures
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|secondary pain amplification
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|Skin disorder
|Skin disorder
|acute cutaneous lupus erythema (ACLE) (also known as “the butterfly rash”)
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*Localized ACLE (ie, malar rash, butterfly rash)
*Generalized ACLE
*Toxic epidermal necrolysis-like ACLE
*Erythema in a malar distribution over the cheeks and nose (but sparing the nasolabial folds), which appears after sun exposure
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|discoid lesions
|more inflammatory and which have a tendency to scar
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|Photosensitivity
|common theme for skin lesions associated with SLE
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|oral and/or nasal ulcers
|usually painless
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|Nonscarring alopecia
|may occur at some point during the course of their disease
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|Subacute cutaneous lupus erythematosus (SCLE)
|Annular SCLE
Papulosquamous SCLE
Drug-induced SCLE
Less common variants: erythrodermic, poikilodermatous, erythema multiforme-like (Rowell syndrome), and vesiculobullous annular SCLE
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|Chronic cutaneous lupus erythematosus (CCLE)
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|Discoid lupus erythematosus (DLE)
Localized DLE


Common initial and chronic complaints are [[fever]], [[malaise]], [[arthralgia|joint pains]], [[myalgia]]s and [[Fatigue (medical)|fatigue]]. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms, however, they are considered suggestive.
Generalized DLE


===Most Common Symptoms===
Hypertrophic DLE
*[[Joint pain]] or [[joint swelling]]
*[[Skin rash]]
*[[Malaise]] or [[fatigue]]


===Complete List Symptoms to look out for===
Lupus erythematosus tumidus (LE tumidus)
*[[Abdominal pain]]
*[[Fatigue]]
*[[Keratoconjunctivitis sicca]]
*[[Leukopenia]]
*[[Pericarditis]]
*[[Pleural effusion]]
*[[Pleuritic chest pain]]
*[[Pneumonitis]]
*[[Polyarthritis]] / [[polyarthralgia]]
*[[Renal disease]]
*[[Renal vasculitis]]
*[[Seizure]]s
*[[Stroke]]
*[[Thrombocytopenia]]
*[[Weight loss]]


===Warning Signs of a Flare===
Lupus profundus (also known as lupus panniculitis)
* Increased [[fatigue]]
*[[Pain]]
*[[Rash]]
*[[Fever]]
*[[Abdominal discomfort]]
*[[Headache]]
*[[Dizziness]]


===Common Symptoms Explained===
Chilblain lupus erythematosus (chilblain LE)
====Dermatological manifestations====
As many as 30% of patients present with some dermatological symptoms (and 65% suffer such symptoms at some point), with 30% to 50% suffering from the classic [[malar rash]] (or ''butterfly rash'') associated with the disease. Patients may present with discoid lupus (thick, red scaly patches on the skin). [[Alopecia]], mouth, nasal, and vaginal [[mouth ulcer|ulcers]], and lesions on the skin are also possible manifestations.


====Musculoskeletal manifestations====
Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome)
Patients most often seek medical attention for [[joint pain]], with small joints of the hand and wrist usually affected, although any joint is at risk. The Lupus Foundation of America "estimates that 95 percent of lupus cases involve achy joints.<ref name="DiGeronimo">DiGeronimo, Theresa. New Hope for People with Lupus. Prima Publishing. 2002.</ref> Unlike [[rheumatoid arthritis]], SLE arthropathy is not usually destructive of [[bone]], however, deformities caused by the disease may become irreversible in as many as 20% of patients.This small percentage may experience "damage to tendons or joint coverings in the hand" which leads to "deformity of the finger joint".<ref name="DiGeronimo">DiGeronimo, Theresa. New Hope for People with Lupus. Prima Publishing. 2002.</ref>
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====Hematological manifestations====
Common initial and chronic complaints are [[fever]], [[malaise]], [[arthralgia|joint pains]], [[myalgia]]s and [[Fatigue (medical)|fatigue]]. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms, however, they are considered suggestive.
[[Anemia]] and iron deficiency may develop in as many as half of patients. Low [[platelet]] and [[white blood cell]] counts may be due to the disease or a side-effect of pharmacological treatment.  Patients may have an association with [[Antiphospholipid syndrome|antiphospholipid antibody syndrome]] (a thrombotic disorder) where autoantibodies to phospholipids are present in the patient's serum.  Abnormalities associated with antiphospholipid antibody syndrome include a paradoxical prolonged PTT (which usually occurs in hemorrhagic disorders) and a positive test for antiphospholipid antibodies; the combination of such findings have earned the term "[[lupus anticoagulant]] positive". Another autoantibody finding in lupus is the [[anticardiolipin antibody]] which can cause a false positive test for [[syphilis]].


====Cardiac manifestations====
===Common Symptoms===
Patients may present with inflammation of various parts of the [[heart]], such as [[pericarditis]], [[myocarditis]], and [[endocarditis]]. The endocarditis of SLE is characteristically non-infective ([[Libman-Sacks endocarditis]]) and involves either the [[mitral valve]] or the [[tricuspid valve]]. [[Atherosclerosis]] also tends to occur more often and advance more rapidly in SLE patients than in the general population.<ref>{{cite journal | author=Yu Asanuma, M.D., Ph.D., Annette Oeser, B.S., Ayumi K. Shintani, Ph.D., M.P.H., Elizabeth Turner, M.D., Nancy Olsen, M.D., Sergio Fazio, M.D., Ph.D., MacRae F. Linton, M.D., Paolo Raggi, M.D., and C. Michael Stein, M.D. | title=Premature coronary-artery atherosclerosis in systemic lupus erythematosus
*Constitutional symptoms
| journal=New England Journal of Medicine | volume=349 | issue=Dec. 18 | year=2003| pages=2407-2414 | url = http://content.nejm.org/cgi/content/full/349/25/2407 | id = PMID 14681506 [http://content.nejm.org/cgi/content/abstract/349/25/2407 Abstract] (full text requires registration) }}</ref><ref>{{cite journal | author=Bevra Hannahs Hahn, M.D. | title=Systemic lupus erythematosus and accelerated atherosclerosis | journal=New England Journal of Medicine | volume=349 | issue=Dec. 18 | year=2003 | pages=2379-2380 | url = http://content.nejm.org/cgi/content/full/349/25/2379 | id =PMID 14681501 [http://content.nejm.org/cgi/content/extract/349/25/2379 Extract] (full text requires registration) }}</ref><ref>{{cite journal | author=Mary J. Roman, M.D., Beth-Ann Shanker, A.B., Adrienne Davis, A.B., Michael D. Lockshin, M.D., Lisa Sammaritano, M.D., Ronit Simantov, M.D., Mary K. Crow, M.D., Joseph E. Schwartz, Ph.D., Stephen A. Paget, M.D., Richard B. Devereux, M.D., and Jane E. Salmon, M.D. | title=Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus  | journal=New England Journal of Medicine | volume=349 | issue=Dec. 18 | year=2003 | pages=2399-2406 | url = http://content.nejm.org/cgi/content/full/349/25/2399
**Fatigue
| id = PMID 14681505 [http://content.nejm.org/cgi/content/abstract/349/25/2399 Abstract] (full text requires registration) }}</ref>
**Fever
**Myalgia
**Muscle tenderness
**Muscle weakness
**Weight change
***Weight gain due to increase in water retention and increase in appetite
***Weight loss due to medication side effect and gastro-intestinal problems
{| class="wikitable"
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| rowspan="6" |Constitutional symptoms
|Fatigue
|the most common complaint 11085805
multidimensional phenomenon due to different factors 7779127


====Pulmonary manifestations====
does not correlate with disease activity 9598886
Lung and pleura inflammation can cause [[Pleurisy|pleuritis]], [[pleural effusion]], lupus pneumonitis, chronic diffuse interstitial lung disease, [[pulmonary hypertension]], [[Pulmonary embolism|pulmonary emboli]], [[pulmonary hemorrhage]].
|frequently associated with depression, sleep disturbances, and concomitant fibromyalgia
|-
|Fever
|a manifestation of active disease-lupus flare, especially within short-term evolution of the disease 14530779
Can be hard to differentiate the cause of fever from other inflammatory and infection diseases


====Hepatic involvement====
Leukopenia or normal range of leukocytes in the setting of fever is more suggestive of lupus activity rather than infection
See [[autoimmune hepatitis]].
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|Myalgia


====Renal involvement====
| rowspan="3" |may be first complaint and initial reason for the patient to seeks medical attention
Painless [[hematuria]] or [[proteinuria]] may often be the only presenting renal symptom. Acute or chronic renal impairment may develop with [[lupus nephritis]], leading to acute or end stage [[renal failure]]. Because of early recognition and management of SLE, end stage renal failure occurs in less than 5% of patients. Histologically, a hallmark of SLE is membranous [[glomerulonephritis]] with "wire loop" abnormalities.<ref>{{cite web |url=http://erl.pathology.iupui.edu/C603/GENE607.HTM |title=General Pathology Images for Immunopathology |accessdate=2007-07-24 |format= |work=}}</ref>  This finding is due to immune complex deposition along the [[glomerular basement membrane]] leading to a typical granular appearance in [[immunofluorescence]] testing.
May happen as a result of treatment with glucocorticoids or glucocorticoid withdrawal
 
| rowspan="3" |perivascular and perifascicular mononuclear cell infiltrates in 25 percent of patients
====Neurological manifestations====
muscle atrophy, microtubular inclusions, mononuclear infiltrate, fiber necrosis, and, occasionally, vacuolated muscle fibers  2319520
About 10% of patients may present with [[seizure]]s or [[psychosis]]. A third may test positive for abnormalities in the [[cerebrospinal fluid]].
|-
 
|Muscle tenderness
====T-cell abnormalities====
|-
Abnormalities in [[T cell]] signaling are associated with SLE, including deficiency in [[CD45]] [[phosphatase]] and increased expression of CD40 ligand.
|Muscle weakness
 
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====Other rarer manifestations====
|Weight change
[[gastroenteritis|Lupus gastroenteritis]], [[pancreatitis|lupus pancreatitis]], [[cystitis|lupus cystitis]], [[autoimmune]] [[Otitis interna|inner ear disease]], [[parasympathetic|parasympathetic dysfunction]], [[vasculitis|retinal vasculitis]], and [[systemic vasculitis|lupus mesenteric vasculitis]].
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*Weight loss due to:
**Decreased appetite
**Side effects of medications (particularly diuretics)
**Gastrointestinal symptoms related to lupus (e.g. gastroesophageal reflux, abdominal pain, peptic ulcer disease, or pancreatitis)
*Weight gain due to:
**Hypoalbuminemia:
***Result in salt and water retention (e.g. due to nephrotic syndrome or protein losing enteropathy)
**Increased appetite associated with the use of glucocorticoids
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|}


==References==
==References==

Revision as of 13:03, 30 June 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Systemic lupus erythematosus (SLE) one of several diseases known as the great imitator[1] because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years.

History and symptoms

Gastrointestinal involvement:

Organ system Disease Sympton
Gastrointestinal involvement Dysphagia 
  • Retrosternal chest pain
  • Heartburn
  • Regurgitation
  • Odynophagia
Peptic ulcer disease
  • Epigastric pain
  • Food-provoked epigastric discomfort and fullness
  • Early satiety
  • Nausea
Intestinal pseudo-obstruction
  • Abdominal pain
  • Bloating
  • Distension
Protein-losing enteropathy
  • Profound edema
  • Hypoalbuminemia
  • Severe diarrhea
Acute pancreatitis
  • Severe persistent epigastric pain often radiating to the back
Mesenteric vasculitis
  • Abdominal pain
  • Food aversion
  • Weight loss
  • Nausea
  • Vomiting
  • Diarrhea
    • Due to chronic mesenteric ischemia
Primary peritonitis
  • Abdominal bloating or distention
  • Nausea and vomiting
  • Diarrhea
  • Constipation or the inability to pass gas
  • Anorexia
Pulmonary involvement Pleural disease
  • Cough
  • Dyspnea
  • Fever
Acute pneumonitis
  • Fever
  • Cough (sometimes with hemoptysis)
  • Dyspnea/ physical examination reveals tachypnea, tachycardia, basilar crackles (may be late inspiratory), and hypoxemia
Pulmonary hemorrhage
  • Dyspnea
  • Cough
  • Hemoptysis
Interstitial lung disease
  • May be asymptomatic
  • Chronic nonproductive cough
  • Dyspnea
  • Decreased exercise tolerance
Thromboembolic disease 
  • Dyspnea
  • Sharp chest pain that may become worse with deep breathing or coughing
Pulmonary hypertension
  • Dyspnea
  • Palpitations
  • Fatigue
  • Impaired exercise tolerance
  • Weakness
  • Syncope
  • Edema
  • Increased abdominal girth/ phE=pulmonary hypertension (loud second heart sound) or cor pulmonale (eg, peripheral edema, ascites, hepatomegaly)/
Shrinking lung
  • Dyspnea
  • Pleuritic chest pain (episodic)
Cardiac involvement Valvular disease
  • most often valve thickening, nodules, and regurgitation, and less frequently vegetations or stenotic lesions, is noted echocardiographically, and the presence of valvular lesions may increase the risk of serious complications
Nonbacterial thrombotic endocarditis (Libman-Sacks, verrucous endocarditis)
  • may occur in patients with SLE and is associated with antiphospholipid antibodies (aPL).
Pericardial disease is noted in approximately one-half of patients with SLE at some time in the course of their disease. Diagnostic pericardiocentesis is suggested for those in whom purulent pericarditis is suspected and for those who do not respond to treatment with nonsteroidal antiinflammatory drugs (NSAIDs) and/or glucocorticoids.
Acute pericarditis Symptomatic often responds to an NSAID; those who do not tolerate or cannot take NSAIDs may use prednisone (0.5 to 1 mg/kg/day in divided doses)
Myocarditis  in SLE can cause resting tachycardia, cardiomegaly, heart failure, conduction abnormalities, and/or arrhythmias.

myocarditis, we suggest initial treatment with high-dose glucocorticoids (Grade 2C). A typical regimen is methylprednisolone 1000 mg intravenously daily for three days.

Coronary heart disease
Neurological involvement Cognitive dysfunction
Stroke mechanisms are heterogenous in SLE and include arterial and venous thrombosis, cardiogenic embolism, and small vessel infarcts
Seizures
Psychosis
  • Hallucinations visual
  • Auditory
Headache
Neuropathies
Genitourinary Nephrotic syndrome
  • Hypertension
  • Peripheral edema
  • Foamy urine
  • Weight gain
Musculoskeletal Arthritis
  • arthralgias
  • effusions
  • Decreased range of motion of both small and large joints
  • Morning stiffness
Avascular necrosis
  • Asymptomatic
  • Mild to moderate pain in groin and lower abdoman
Bone fragility fractures
Secondary pain amplification
Avascular necrosis can occur in patients treated with corticosteroids
Osteoporosis 
  • Loss of height
  • Sudden back pain
Skin disorder acute cutaneous lupus erythema (ACLE) (also known as “the butterfly rash”)
  • Localized ACLE (ie, malar rash, butterfly rash)
  • Generalized ACLE
  • Toxic epidermal necrolysis-like ACLE
  • Erythema in a malar distribution over the cheeks and nose (but sparing the nasolabial folds), which appears after sun exposure
discoid lesions more inflammatory and which have a tendency to scar
Photosensitivity common theme for skin lesions associated with SLE
oral and/or nasal ulcers usually painless
Nonscarring alopecia may occur at some point during the course of their disease
Subacute cutaneous lupus erythematosus (SCLE) Annular SCLE

Papulosquamous SCLE

Drug-induced SCLE

Less common variants: erythrodermic, poikilodermatous, erythema multiforme-like (Rowell syndrome), and vesiculobullous annular SCLE

Chronic cutaneous lupus erythematosus (CCLE) Discoid lupus erythematosus (DLE)

Localized DLE

Generalized DLE

Hypertrophic DLE

Lupus erythematosus tumidus (LE tumidus)

Lupus profundus (also known as lupus panniculitis)

Chilblain lupus erythematosus (chilblain LE)

Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome)

Common initial and chronic complaints are fever, malaise, joint pains, myalgias and fatigue. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms, however, they are considered suggestive.

Common Symptoms

  • Constitutional symptoms
    • Fatigue
    • Fever
    • Myalgia
    • Muscle tenderness
    • Muscle weakness
    • Weight change
      • Weight gain due to increase in water retention and increase in appetite
      • Weight loss due to medication side effect and gastro-intestinal problems
Constitutional symptoms Fatigue the most common complaint 11085805

multidimensional phenomenon due to different factors 7779127

does not correlate with disease activity 9598886

frequently associated with depression, sleep disturbances, and concomitant fibromyalgia
Fever a manifestation of active disease-lupus flare, especially within short-term evolution of the disease 14530779

Can be hard to differentiate the cause of fever from other inflammatory and infection diseases

Leukopenia or normal range of leukocytes in the setting of fever is more suggestive of lupus activity rather than infection

Myalgia may be first complaint and initial reason for the patient to seeks medical attention

May happen as a result of treatment with glucocorticoids or glucocorticoid withdrawal

perivascular and perifascicular mononuclear cell infiltrates in 25 percent of patients

muscle atrophy, microtubular inclusions, mononuclear infiltrate, fiber necrosis, and, occasionally, vacuolated muscle fibers 2319520

Muscle tenderness
Muscle weakness
Weight change
  • Weight loss due to:
    • Decreased appetite
    • Side effects of medications (particularly diuretics)
    • Gastrointestinal symptoms related to lupus (e.g. gastroesophageal reflux, abdominal pain, peptic ulcer disease, or pancreatitis)
  • Weight gain due to:
    • Hypoalbuminemia:
      • Result in salt and water retention (e.g. due to nephrotic syndrome or protein losing enteropathy)
    • Increased appetite associated with the use of glucocorticoids

References

  1. ↑ Lupus: The Great Imitator

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