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==Overview==
==Overview==
[[Sarcoidosis]] is a multisystem [[granulomatous]] disorder of unknown etiology that can involve all organ systems to varying extent and degree.
==History and Symptoms==
==History and Symptoms==
Sarcoidosis is a Systemic Disease that can affect any organ. Common symptoms are vague, such as [[fatigue (physical)|fatigue]] unchanged by sleep, [[asthenia|lack of energy]], [[weight loss]], aches and pains, [[arthralgia]], [[xerophthalmia|dry eyes]], blurry vision, [[dyspnea|shortness of breath]], a dry hacking [[cough]] or skin lesions. The cutaneous symptoms vary, and range from [[rash]]es and noduli (small bumps) to [[erythema nodosum]] or [[lupus pernio]]. It is often [[asymptomatic]].
===[[Pulmonary Sarcoidosis]]===
* [[Sarcoidosis]] commonly involves patients between 20 and 60 years of age<ref>Judson MA, Boan AD, Lackland DT: The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2012, 29(2):119-127.</ref>. Lung is the most common organ involved by [[sarcoidosis]], but up to 30% of patients present with [[extrapulmonary sarcoidosis]]<ref>Ungprasert P, Carmona EM, Utz JP, Ryu JH, Crowson CS, Matteson EL: Epidemiology of Sarcoidosis 1946-2013: A Population-Based Study. Mayo Clinic proceedings 2016, 91(2):183-188.</ref><ref>Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Jr., Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ et al: Clinical characteristics of patients in a case control study of sarcoidosis. American journal of respiratory and critical care medicine 2001, 164(10 Pt 1):1885-1889.</ref>. [[Pulmonary sarcoidosis]] commonly presents with [[cough]], [[dyspnea]], [[chest pain]], and infrequently [[fatigue]], [[malaise]], [[fever]], and [[weight loss]]<ref>Sharma OP: Fatigue and sarcoidosis. The European respiratory journal 1999, 13(4):713-714.</ref>.
* Young children can present with [[skin rash]], [[arthritis]], [[uveitis]], and [[erythema nodosum]], without a predominant lung involvement<ref>Pattishall EN, Kendig EL, Jr.: Sarcoidosis in children. Pediatric pulmonology 1996, 22(3):195-203.</ref><ref>Milman N, Hoffmann AL: Childhood sarcoidosis: long-term follow-up. The European respiratory journal 2008, 31(3):592-598.</ref>
 
===[[Cutaneous Sarcoidosis]]===
In 25% of the patients diagnosed with [[sarcoidosis]], cutaneous involvement is seen<ref>Roberts SD, Mirowski GW, Wilkes D, Kwo PY, Knox KS: Sarcoidosis. Part II: extrapulmonary and systemic manifestations. Journal of the American Academy of Dermatology 2004, 51(4):628-630.</ref>. The most common patterns of involvement is: papular, nodular, [[plaque-like]], [[lupus pernio]], [[erythema nodosum]], subcutaneous sarcoidosis. See cutaneous manifestations of [[sarcoidosis]] in physical exam for more detail.
 
===[[Ocular Sarcoidosis]]===
 
In 25% of the patients diagnosed with [[sarcoidosis]], ocular involvement is seen<ref>Jamilloux Y, Kodjikian L, Broussolle C, Seve P: Sarcoidosis and uveitis. Autoimmunity reviews 2014, 13(8):840-849.</ref>. [[Sarcoidosis]] involves the [[orbit]], [[anterior segment]], [[posterior segment]], [[conjunctiva]], [[lacrimal glands]], and [[extraocular muscles]]. the usual symptoms include [[dry eye]], [[blurred vision]], [[photophobia]], [[red eye]], and pain<ref name="uv">Evans M, Sharma O, LaBree L, Smith RE, Rao NA: Differences in clinical findings between Caucasians and African Americans with biopsy-proven sarcoidosis. Ophthalmology 2007, 114(2):325-333</ref><ref>Birnbaum AD, French DD, Mirsaeidi M, Wehrli S: Sarcoidosis in the national veteran population: association of ocular inflammation and mortality. Ophthalmology 2015, 122(5):934-938.</ref> .
*'''Intraocular sarcoidosis''': these include anterior, intermediate, and posterior [[uveitis]]. Typical symptoms of anterior [[uveitis]] are pain and redness perticularly at the junction between [[cornea]] and [[sclera]] ([[limbus]]), while posterior and/or intermediate [[uveitis]] causes no symptom or [[floaters]]<ref name="uv">Evans M, Sharma O, LaBree L, Smith RE, Rao NA: Differences in clinical findings between Caucasians and African Americans with biopsy-proven sarcoidosis. Ophthalmology 2007, 114(2):325-333</ref>.
*'''extraocular orbital sarcoidosis''': involvement of [[lacrimal glands]], [[conjunctiva]], [[extraocular muscles]], [[optic nerve]] and palpable mass<ref>Mavrikakis I, Rootman J: Diverse clinical presentations of orbital sarcoid. American journal of ophthalmology 2007, 144(5):769-775.</ref>.
 
===Upper Respiratory Tract Disease===
 
[[Sarcoidosis]] can involve the [[larynx]], [[pharynx]], [[nares]], and sinuses. The symptoms can include [[cough]], [[dysphagia]], [[hoarsness]], [[nasal obstruction]], [[nasal crusting]], [[anosmia]], [[epistaxis]], and [[nasal polyposis]]<ref>Baughman RP, Lower EE, Tami T: Upper airway. 4: Sarcoidosis of the upper respiratory tract (SURT). Thorax 2010, 65(2):181-186.</ref><ref>Reed J, deShazo RD, Houle TT, Stringer S, Wright L, Moak JS, 3rd: Clinical features of sarcoid rhinosinusitis. The American journal of medicine 2010, 123(9):856-862.</ref>.
 
===[[Cardiac Sarcoidosis]]===
 
The exact rate of myocardial involvement in [[sarcoidosis]] is not clear, but it ranges from 5% in patients with systemic [[sarcoidosis]] to 25 and even 70% in autopsy studies. Patients may complain of [[palpitation]]s, [[syncope]], [[lightheadedness]], and [[chest pain]]. [[sudden cardiac death]] ([[SCD]]) can occur<ref>Kron J, Ellenbogen KA: Cardiac sarcoidosis: contemporary review. Journal of cardiovascular electrophysiology 2015, 26(1):104-109.</ref><ref>Darlington P, Gabrielsen A, Sorensson P, Cederlund K, Eklund A, Grunewald J: Cardiac involvement in Caucasian patients with pulmonary sarcoidosis. Respiratory research 2014, 15:15</ref>.
 
===Neurosarcoidosis===
Sarcoidosis can involve both Central and Peripheral nervous system in 5% of patients. The common symptoms include:
* Peripheral [[facial nerve palsy]] in 25 to 50% of patients with neurosarcoidosis<ref>Stern BJ, Krumholz A, Johns C, Scott P, Nissim J: Sarcoidosis and its neurological manifestations. Archives of neurology 1985, 42(9):909-917.</ref><ref name="sl">Joseph FG, Scolding NJ: Neurosarcoidosis: a study of 30 new cases. Journal of neurology, neurosurgery, and psychiatry 2009, 80(3):297-304.</ref>
* [[Polyuria]] and impairment in sleep, [[thirst]], [[body temprature]] and [[libido]] due to hypothalamic inflammation<ref name="sl">Joseph FG, Scolding NJ: Neurosarcoidosis: a study of 30 new cases. Journal of neurology, neurosurgery, and psychiatry 2009, 80(3):297-304.</ref><ref>Bihan H, Christozova V, Dumas JL, Jomaa R, Valeyre D, Tazi A, Reach G, Krivitzky A, Cohen R: Sarcoidosis: clinical, hormonal, and magnetic resonance imaging (MRI) manifestations of hypothalamic-pituitary disease in 9 patients and review of the literature. Medicine 2007, 86(5):259-268.</ref>
* Seizures<ref>Pawate S, Moses H, Sriram S: Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM : monthly journal of the Association of Physicians 2009, 102(7):449-460.</ref>
 
 
 
 
===Reticuloendothelial System===
Involvement of the [[lymph node]]s, [[liver]], and [[spleen]] is common in [[sarcoidosis]]. The patients may present with neck or axillary mass. liver and spleen involvement is usually asymptomatic<ref>Warshauer DM, Lee JK: Imaging manifestations of abdominal sarcoidosis. AJR American journal of roentgenology 2004, 182(1):15-28.</ref>.
 
===Musculocutaneous===
There are 3 common patterns of musculocutaneous involvement in sarcoidosis: 1. [[Acute sarcoid arthritis]] or acute polyarthritis 2. Chronic arthritis 3. [[Myopathy]]([[diffuse granulomatous myositis]])<ref>Kellner H, Spathling S, Herzer P: Ultrasound findings in Lofgren's syndrome: is ankle swelling caused by arthritis, tenosynovitis or periarthritis? The Journal of rheumatology 1992, 19(1):38-41.</ref>.
 
===[[Lofgren Syndrome]]===
The presence of [[erythema nodosum]], [[hilar lymphadenopathy]], [[migratory polyarthralgia]], and [[fever]] has a 95% diagnostic [[specifity]] for [[sarcoidosis]], allowing a diagnosis to be made without biopsy, and is associated with a good prognosis<ref>O'Regan A, Berman JS: Sarcoidosis. Annals of internal medicine 2012, 156(9):ITC5-1, ITC5-2, ITC5-3, ITC5-4, ITC5-5, ITC5-6, ITC5-7, ITC5-8, ITC5-9, ITC5-10, ITC15-11, ITC15-12, ITC15-13, ITC15-14, ITC15-15; quiz ITC15-16.</ref>.
 
===Exocrine Glands===
In 5% of the patients diagnosed with [[sarcoidosis]], painless swelling of [[salivary glands]] and [[parotid]] gland occurs. [[Sicca syndrome]] and [[xerostomia]] may also be seen<ref>James DG, Sharma OP: Parotid gland sarcoidosis. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2000, 17(1):27-32.</ref>.
 
===Renal & Electrolyte===
* Symptoms caused by [[hypercalcemia]] (presenting feature in 10 to 20 percent of patients)
* Renal colic caused by [[nephrolithiasis]] (presenting feature 4 percent of patients)
* Symptoms caused by uremia due to [[interstitial nephritis]]
* Symptoms caused by [[proteinuria]] (various types of glumerulonephritis may be seen)<ref>Rizzato G, Colombo P: Nephrolithiasis as a presenting feature of chronic sarcoidosis: a prospective study. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 1996, 13(2):167-172.</ref><ref>presentation of sarcoidosis: a frequent occurrence but a challenge to an early diagnosis. A 3-year-long prospective observational study. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2004, 21(2):119-126</ref>.
 


The combination of [[erythema nodosum]], bilateral [[hilar]] [[lymphadenopathy]] and [[arthralgia]] is called [[Lofgren syndrome|''Lofgren syndrome'']]. This syndrome has a relatively good prognosis.


Renal, liver (including [[portal hypertension]]), heart or brain involvement may cause further symptoms and altered functioning. Manifestations in the eye include [[uveitis]] and retinal inflammation, which may result in loss of visual acuity or blindness. Sarcoidosis affecting the brain or nerves is known as [[neurosarcoidosis]].


The combination of [[anterior uveitis]], [[parotitis]] and fever is called [[uveoparotitis]], and is associated with ''Heerfordt-Waldenstrom syndrome''. ({{ICD10|D|86|8|d|80}})


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}


[[Category:Ailments of unknown etiology]]
[[Category:Pulmonology]]
[[Category:Pulmonology]]
[[Category:Abdominal pain]]
[[Category:Rheumatology]]
[[Category:Rheumatology]]


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Latest revision as of 22:12, 14 May 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Roshan Dinparasti Saleh M.D.

Overview

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology that can involve all organ systems to varying extent and degree.

History and Symptoms

Pulmonary Sarcoidosis

Cutaneous Sarcoidosis

In 25% of the patients diagnosed with sarcoidosis, cutaneous involvement is seen[7]. The most common patterns of involvement is: papular, nodular, plaque-like, lupus pernio, erythema nodosum, subcutaneous sarcoidosis. See cutaneous manifestations of sarcoidosis in physical exam for more detail.

Ocular Sarcoidosis

In 25% of the patients diagnosed with sarcoidosis, ocular involvement is seen[8]. Sarcoidosis involves the orbit, anterior segment, posterior segment, conjunctiva, lacrimal glands, and extraocular muscles. the usual symptoms include dry eye, blurred vision, photophobia, red eye, and pain[9][10] .

Upper Respiratory Tract Disease

Sarcoidosis can involve the larynx, pharynx, nares, and sinuses. The symptoms can include cough, dysphagia, hoarsness, nasal obstruction, nasal crusting, anosmia, epistaxis, and nasal polyposis[12][13].

Cardiac Sarcoidosis

The exact rate of myocardial involvement in sarcoidosis is not clear, but it ranges from 5% in patients with systemic sarcoidosis to 25 and even 70% in autopsy studies. Patients may complain of palpitations, syncope, lightheadedness, and chest pain. sudden cardiac death (SCD) can occur[14][15].

Neurosarcoidosis

Sarcoidosis can involve both Central and Peripheral nervous system in 5% of patients. The common symptoms include:



Reticuloendothelial System

Involvement of the lymph nodes, liver, and spleen is common in sarcoidosis. The patients may present with neck or axillary mass. liver and spleen involvement is usually asymptomatic[20].

Musculocutaneous

There are 3 common patterns of musculocutaneous involvement in sarcoidosis: 1. Acute sarcoid arthritis or acute polyarthritis 2. Chronic arthritis 3. Myopathy(diffuse granulomatous myositis)[21].

Lofgren Syndrome

The presence of erythema nodosum, hilar lymphadenopathy, migratory polyarthralgia, and fever has a 95% diagnostic specifity for sarcoidosis, allowing a diagnosis to be made without biopsy, and is associated with a good prognosis[22].

Exocrine Glands

In 5% of the patients diagnosed with sarcoidosis, painless swelling of salivary glands and parotid gland occurs. Sicca syndrome and xerostomia may also be seen[23].

Renal & Electrolyte



References

  1. ↑ Judson MA, Boan AD, Lackland DT: The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2012, 29(2):119-127.
  2. ↑ Ungprasert P, Carmona EM, Utz JP, Ryu JH, Crowson CS, Matteson EL: Epidemiology of Sarcoidosis 1946-2013: A Population-Based Study. Mayo Clinic proceedings 2016, 91(2):183-188.
  3. ↑ Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H, Jr., Bresnitz EA, DePalo L, Hunninghake G, Iannuzzi MC, Johns CJ et al: Clinical characteristics of patients in a case control study of sarcoidosis. American journal of respiratory and critical care medicine 2001, 164(10 Pt 1):1885-1889.
  4. ↑ Sharma OP: Fatigue and sarcoidosis. The European respiratory journal 1999, 13(4):713-714.
  5. ↑ Pattishall EN, Kendig EL, Jr.: Sarcoidosis in children. Pediatric pulmonology 1996, 22(3):195-203.
  6. ↑ Milman N, Hoffmann AL: Childhood sarcoidosis: long-term follow-up. The European respiratory journal 2008, 31(3):592-598.
  7. ↑ Roberts SD, Mirowski GW, Wilkes D, Kwo PY, Knox KS: Sarcoidosis. Part II: extrapulmonary and systemic manifestations. Journal of the American Academy of Dermatology 2004, 51(4):628-630.
  8. ↑ Jamilloux Y, Kodjikian L, Broussolle C, Seve P: Sarcoidosis and uveitis. Autoimmunity reviews 2014, 13(8):840-849.
  9. ↑ 9.0 9.1 Evans M, Sharma O, LaBree L, Smith RE, Rao NA: Differences in clinical findings between Caucasians and African Americans with biopsy-proven sarcoidosis. Ophthalmology 2007, 114(2):325-333
  10. ↑ Birnbaum AD, French DD, Mirsaeidi M, Wehrli S: Sarcoidosis in the national veteran population: association of ocular inflammation and mortality. Ophthalmology 2015, 122(5):934-938.
  11. ↑ Mavrikakis I, Rootman J: Diverse clinical presentations of orbital sarcoid. American journal of ophthalmology 2007, 144(5):769-775.
  12. ↑ Baughman RP, Lower EE, Tami T: Upper airway. 4: Sarcoidosis of the upper respiratory tract (SURT). Thorax 2010, 65(2):181-186.
  13. ↑ Reed J, deShazo RD, Houle TT, Stringer S, Wright L, Moak JS, 3rd: Clinical features of sarcoid rhinosinusitis. The American journal of medicine 2010, 123(9):856-862.
  14. ↑ Kron J, Ellenbogen KA: Cardiac sarcoidosis: contemporary review. Journal of cardiovascular electrophysiology 2015, 26(1):104-109.
  15. ↑ Darlington P, Gabrielsen A, Sorensson P, Cederlund K, Eklund A, Grunewald J: Cardiac involvement in Caucasian patients with pulmonary sarcoidosis. Respiratory research 2014, 15:15
  16. ↑ Stern BJ, Krumholz A, Johns C, Scott P, Nissim J: Sarcoidosis and its neurological manifestations. Archives of neurology 1985, 42(9):909-917.
  17. ↑ 17.0 17.1 Joseph FG, Scolding NJ: Neurosarcoidosis: a study of 30 new cases. Journal of neurology, neurosurgery, and psychiatry 2009, 80(3):297-304.
  18. ↑ Bihan H, Christozova V, Dumas JL, Jomaa R, Valeyre D, Tazi A, Reach G, Krivitzky A, Cohen R: Sarcoidosis: clinical, hormonal, and magnetic resonance imaging (MRI) manifestations of hypothalamic-pituitary disease in 9 patients and review of the literature. Medicine 2007, 86(5):259-268.
  19. ↑ Pawate S, Moses H, Sriram S: Presentations and outcomes of neurosarcoidosis: a study of 54 cases. QJM : monthly journal of the Association of Physicians 2009, 102(7):449-460.
  20. ↑ Warshauer DM, Lee JK: Imaging manifestations of abdominal sarcoidosis. AJR American journal of roentgenology 2004, 182(1):15-28.
  21. ↑ Kellner H, Spathling S, Herzer P: Ultrasound findings in Lofgren's syndrome: is ankle swelling caused by arthritis, tenosynovitis or periarthritis? The Journal of rheumatology 1992, 19(1):38-41.
  22. ↑ O'Regan A, Berman JS: Sarcoidosis. Annals of internal medicine 2012, 156(9):ITC5-1, ITC5-2, ITC5-3, ITC5-4, ITC5-5, ITC5-6, ITC5-7, ITC5-8, ITC5-9, ITC5-10, ITC15-11, ITC15-12, ITC15-13, ITC15-14, ITC15-15; quiz ITC15-16.
  23. ↑ James DG, Sharma OP: Parotid gland sarcoidosis. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2000, 17(1):27-32.
  24. ↑ Rizzato G, Colombo P: Nephrolithiasis as a presenting feature of chronic sarcoidosis: a prospective study. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 1996, 13(2):167-172.
  25. ↑ presentation of sarcoidosis: a frequent occurrence but a challenge to an early diagnosis. A 3-year-long prospective observational study. Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG 2004, 21(2):119-126

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