Difference between revisions of "Sarcoidosis"

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Sarcoidosis (view source)

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{{ Infobox diagnosis
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Name      = {{PAGENAME}}
| Image      = Sarcoidosis (1) lymph node biopsy.jpg
| Image      = Sarcoidosis (1) lymph node biopsy.jpg  
| Width      =
| Width      =
| Caption    = Sarcoidosis-like granulomas in a lymph node. [[H&E stain]].
| Caption    = Sarcoidosis-like granulomas in a lymph node. [[H&E stain]].
| Micro      =
| Micro      = well-formed [[granuloma]]s often with few surrounding lymphocytes ("naked"), usually non-necrotizing
| Subtypes  =
| Subtypes  =
| LMDDx      =
| LMDDx      = fungal infections, [[MAC]], [[tuberculosis]], other infections, drug reactions, reactive process to malignancy
| Stains    =
| Stains    = AFB -ve, GMS -ve, PASD -ve
| IHC        =
| IHC        =  
| EM        =
| EM        =
| Molecular  =
| Molecular  = PCR for [[tuberculosis]] -ve
| IF        =
| IF        =
| Gross      =  
| Gross      =  
| Grossing  =
| Grossing  =
| Site      = hilar lymph nodes (lung), other sites
| Site      = [[lung]], hilar lymph nodes of the lung, [[skin]], [[cardiac sarcoidosis|heart]], other sites
| Assdx      =
| Assdx      =
| Syndromes  =
| Syndromes  =
Line 21: Line 21:
| Symptoms  =
| Symptoms  =
| Prevalence = uncommon
| Prevalence = uncommon
| Bloodwork  =
| Bloodwork  = +/-ACE elevated
| Rads      = bilateral hilar lymphadenopathy
| Rads      = +/-bilateral hilar lymphadenopathy (very common), +/-interstitial pattern, +/- pulmonary infiltrates, +/-cystic/bullous changes
| Endoscopy  =
| Endoscopy  =
| Prognosis  =
| Prognosis  =
| Other      =
| Other      =  
| ClinDDx    =
| ClinDDx    = [[lymphoma]], [[metastatic]] carcinoma, [[Wegener's granulomatosis]], others
}}
}}
'''Sarcoidosis''' is non-necrotizing granulomatous disease of unknown etiology.  It classically associated with (pulmonary) hilar lymphadenopathy.  It may be found in almost any organ, e.g. [[heart]], [[appendix]].
'''Sarcoidosis''' is non-necrotizing [[granulomatous disease]] of unknown etiology.  It classically associated with (pulmonary) hilar lymphadenopathy.  It may be found in almost any organ.
 
This article covers the topic in general and focuses on the [[lung]] aspects. [[Cardiac sarcoidosis]] is dealt with separately.


==General==
==General==
*Diagnosis of exclusion - infection must be excluded.
*[[Diagnosis]] of exclusion - infection, neoplasm, and drugs must be excluded.
*Rare.
*Uncommon.
*Afflicits skin ~25% of the time.<ref name=pmid24138972>{{Cite journal  | last1 = Noiles | first1 = K. | last2 = Beleznay | first2 = K. | last3 = Crawford | first3 = RI. | last4 = Au | first4 = S. | title = Sarcoidosis can present with necrotizing granulomas histologically: two cases of ulcerated sarcoidosis and review of the literature. | journal = J Cutan Med Surg | volume = 17 | issue = 6 | pages = 377-83 | month =  | year =  | doi =  | PMID = 24138972 }}</ref>
 
Serology:
*Angiotensin-converting enzyme (ACE) - used for diagnosis and to monitor activity.<ref name=pmid24047001>{{Cite journal  | last1 = Kaura | first1 = V. | last2 = Kaura | first2 = NV. | last3 = Kaura | first3 = BN. | last4 = Kaura | first4 = CS. | title = Angiotensin-converting enzyme inhibitors in the treatment of sarcoidosis and association with ACE gene polymorphism: case series. | journal = Indian J Chest Dis Allied Sci | volume = 55 | issue = 2 | pages = 105-7 | month =  | year =  | doi =  | PMID = 24047001 }}</ref><ref name=pmid23897103>{{Cite journal  | last1 = Stouten | first1 = K. | last2 = Werken | first2 = MV. | last3 = Tchetverikov | first3 = I. | last4 = Saboerali | first4 = M. | last5 = Vermeer | first5 = HJ. | last6 = Castel | first6 = R. | last7 = Verheijen | first7 = FM. | title = Extreme elevation of serum angiotensin-converting enzyme (ACE) activity: always consider familial ACE hyperactivity. | journal = Ann Clin Biochem | volume =  | issue =  | pages =  | month = Jul | year = 2013 | doi = 10.1177/0004563213489812 | PMID = 23897103 }}</ref>
**Elevated in approximately 65% of patients in one series.<ref name=pmid9327039>{{Cite journal  | last1 = Shorr | first1 = AF. | last2 = Torrington | first2 = KG. | last3 = Parker | first3 = JM. | title = Serum angiotensin converting enzyme does not correlate with radiographic stage at initial diagnosis of sarcoidosis. | journal = Respir Med | volume = 91 | issue = 7 | pages = 399-401 | month = Aug | year = 1997 | doi =  | PMID = 9327039 }}</ref>


==Gross==
==Gross==
*Bilateral lymphadenopathy ~90% of cases.
*Lungs - classic location.<ref name=pmid23597964>{{Cite journal  | last1 = Rao | first1 = DA. | last2 = Dellaripa | first2 = PF. | title = Extrapulmonary manifestations of sarcoidosis. | journal = Rheum Dis Clin North Am | volume = 39 | issue = 2 | pages = 277-97 | month = May | year = 2013 | doi = 10.1016/j.rdc.2013.02.007 | PMID = 23597964 }}
</ref>
*Bilateral hilar lymphadenopathy.
 
DDx lungs - radiologic:
*Carcinomatosis - interstitial pattern.<ref>URL: [http://www.radiologyassistant.nl/en/46b480a6e4bdc http://www.radiologyassistant.nl/en/46b480a6e4bdc]. Accessed on: 23 May 2010.</ref>
*Lymphoma - bilateral lymphadenopathy +/- mediastinal lymphadenopathy.<ref name=pmid23207258>{{Cite journal  | last1 = Boujaoude | first1 = Z. | last2 = Dahdel | first2 = M. | last3 = Pratter | first3 = M. | last4 = Kass | first4 = J. | title = Endobronchial ultrasound with transbronchial needle aspiration in the diagnosis of bilateral hilar and mediastinal lymphadenopathy. | journal = J Bronchology Interv Pulmonol | volume = 19 | issue = 1 | pages = 19-23 | month = Jan | year = 2012 | doi = 10.1097/LBR.0b013e3182442b89 | PMID = 23207258 }}</ref>


Note:
<gallery>
*Radiologic differential diagnosis includes carcinomatosis.<ref>URL: [http://www.radiologyassistant.nl/en/46b480a6e4bdc http://www.radiologyassistant.nl/en/46b480a6e4bdc]. Accessed on: 23 May 2010.</ref>
File:Sarcoidosis - Bilateral hilar lymphadenoathy (6076995153).jpg | Markedly enlarged bilaterally hilar lymph nodes in stage 1 sarcoidosis (WC/Yale Rosen)
File:Sarcoidosis - Fibrosis (6076822244).jpg | Fibrosis (WC/Yale Rosen)
</gallery>


==Microscopic==
==Microscopic==
Features:
Features:
*[[Granulomata]], well-formed, non-necrotizing.
*[[Granulomata]], well-formed, non-necrotizing. ‡
**Negative for microorganisms with special stains ([[PAS-D]], [[GMS]], [[AFB]]).
**Negative for microorganisms with special stains.
*Usu. minimal (lymphoid) inflammation; sarcoid granulomas are known as "naked granulomas".<ref name=pmid18948765>{{Cite journal  | last1 = Brinster | first1 = NK. | title = Dermatopathology for the surgical pathologist: a pattern-based approach to the diagnosis of inflammatory skin disorders (part II). | journal = Adv Anat Pathol | volume = 15 | issue = 6 | pages = 350-69 | month = Nov | year = 2008 | doi = 10.1097/PAP.0b013e31818b1ac6 | PMID = 18948765 }}</ref>
*Usu. minimal (lymphoid) inflammation; sarcoid granulomas are known as "naked granulomas".<ref name=pmid18948765>{{Cite journal  | last1 = Brinster | first1 = NK. | title = Dermatopathology for the surgical pathologist: a pattern-based approach to the diagnosis of inflammatory skin disorders (part II). | journal = Adv Anat Pathol | volume = 15 | issue = 6 | pages = 350-69 | month = Nov | year = 2008 | doi = 10.1097/PAP.0b013e31818b1ac6 | PMID = 18948765 }}</ref><ref name=pmid24138972>{{Cite journal  | last1 = Noiles | first1 = K. | last2 = Beleznay | first2 = K. | last3 = Crawford | first3 = RI. | last4 = Au | first4 = S. | title = Sarcoidosis can present with necrotizing granulomas histologically: two cases of ulcerated sarcoidosis and review of the literature. | journal = J Cutan Med Surg | volume = 17 | issue = 6 | pages = 377-83 | month =  | year =  | doi =  | PMID = 24138972 }}</ref>
*In lung: interstitial location.
*In lung:  
**Interstitial location +/- centrilobular.
 
Notes:
*‡ Reported with necrosis - uncommon.<ref name=pmid24138972>{{Cite journal  | last1 = Noiles | first1 = K. | last2 = Beleznay | first2 = K. | last3 = Crawford | first3 = RI. | last4 = Au | first4 = S. | title = Sarcoidosis can present with necrotizing granulomas histologically: two cases of ulcerated sarcoidosis and review of the literature. | journal = J Cutan Med Surg | volume = 17 | issue = 6 | pages = 377-83 | month =  | year =  | doi =  | PMID = 24138972 }}</ref>


DDx:
DDx:
*Reactive changes - may mimic sarcoidosis.
*Infections.
*Drug reaction.
**Fungal.
**Mycobacterial, e.g. [[tuberculosis]].
**Tertiary [[syphilis]].<ref name=pmid19249139>{{Cite journal  | last1 = Hervier | first1 = B. | last2 = Wastiaux | first2 = H. | last3 = Freour | first3 = T. | last4 = Masseau | first4 = A. | last5 = Corvec | first5 = S. | last6 = Armingeat | first6 = T. | last7 = Hamidou | first7 = M. | title = [Sarcoidosis-like granulomatosis revealing a tertiary syphilis]. | journal = Rev Med Interne | volume = 30 | issue = 9 | pages = 806-8 | month = Sep | year = 2009 | doi = 10.1016/j.revmed.2009.01.003 | PMID = 19249139 }}</ref>
*[[Hypersensitivity pneumonitis]] - usually poorly-formed granulomas, centrilobular, not paraseptal.{{fact}}
*Reaction to treatment/drug.
**Anti-TNF therapy.<ref name=pmid22389903>{{Cite journal  | last1 = Tong | first1 = D. | last2 = Manolios | first2 = N. | last3 = Howe | first3 = G. | last4 = Spencer | first4 = D. | title = New onset sarcoid-like granulomatosis developing during anti-TNF therapy: an under-recognised complication. | journal = Intern Med J | volume = 42 | issue = 1 | pages = 89-94 | month = Jan | year = 2012 | doi =  | PMID = 22389903 }}</ref>
**Anti-TNF therapy.<ref name=pmid22389903>{{Cite journal  | last1 = Tong | first1 = D. | last2 = Manolios | first2 = N. | last3 = Howe | first3 = G. | last4 = Spencer | first4 = D. | title = New onset sarcoid-like granulomatosis developing during anti-TNF therapy: an under-recognised complication. | journal = Intern Med J | volume = 42 | issue = 1 | pages = 89-94 | month = Jan | year = 2012 | doi =  | PMID = 22389903 }}</ref>
**Ipilimumab.<ref name=pmid24124863>{{Cite journal  | last1 = Reule | first1 = RB. | last2 = North | first2 = JP. | title = Cutaneous and pulmonary sarcoidosis-like reaction associated with ipilimumab. | journal = J Am Acad Dermatol | volume = 69 | issue = 5 | pages = e272-3 | month = Nov | year = 2013 | doi = 10.1016/j.jaad.2013.07.028 | PMID = 24124863 }}</ref>
**Ipilimumab.<ref name=pmid24124863>{{Cite journal  | last1 = Reule | first1 = RB. | last2 = North | first2 = JP. | title = Cutaneous and pulmonary sarcoidosis-like reaction associated with ipilimumab. | journal = J Am Acad Dermatol | volume = 69 | issue = 5 | pages = e272-3 | month = Nov | year = 2013 | doi = 10.1016/j.jaad.2013.07.028 | PMID = 24124863 }}</ref>
*Infections.
*[[Common variable immunodeficiency]].<ref name=pmid18496983>{{Cite journal  | last1 = Vultaggio | first1 = A. | last2 = Matucci | first2 = A. | last3 = Parronchi | first3 = P. | last4 = Rossi | first4 = O. | last5 = Filì | first5 = L. | last6 = Giudizi | first6 = MG. | last7 = Palandri | first7 = F. | last8 = Agostini | first8 = C. | last9 = Semenzato | first9 = G. | title = Association between sarcoidosis-like disease and common variable immunodeficiency (CVI): a new CVI variant showing an activation of the immune system. | journal = Sarcoidosis Vasc Diffuse Lung Dis | volume = 24 | issue = 2 | pages = 127-33 | month = Sep | year = 2007 | doi = | PMID = 18496983 }}</ref>
**[[Syphilis]].<ref name=pmid19249139>{{Cite journal  | last1 = Hervier | first1 = B. | last2 = Wastiaux | first2 = H. | last3 = Freour | first3 = T. | last4 = Masseau | first4 = A. | last5 = Corvec | first5 = S. | last6 = Armingeat | first6 = T. | last7 = Hamidou | first7 = M. | title = [Sarcoidosis-like granulomatosis revealing a tertiary syphilis]. | journal = Rev Med Interne | volume = 30 | issue = 9 | pages = 806-8 | month = Sep | year = 2009 | doi = 10.1016/j.revmed.2009.01.003 | PMID = 19249139 }}</ref>
*Reactive changes associated with tumours:
*[[Common variable immunodeficiency]].<ref name=pmid18496983>{{Cite journal  | last1 = Vultaggio | first1 = A. | last2 = Matucci | first2 = A. | last3 = Parronchi | first3 = P. | last4 = Rossi | first4 = O. | last5 = Filì | first5 = L. | last6 = Giudizi | first6 = MG. | last7 = Palandri | first7 = F. | last8 = Agostini | first8 = C. | last9 = Semenzato | first9 = G. | title = Association between sarcoidosis-like disease and common variable immunodeficiency (CVI): a new CVI variant showing an activation of the immune system. | journal = Sarcoidosis Vasc Diffuse Lung Dis | volume = 24 | issue = 2 | pages = 127-33 | month = Sep | year = 2007 | doi =  | PMID = 18496983 }}
**All testicular tumours<ref name=pmid18496978>{{Cite journal  | last1 = Paparel | first1 = P. | last2 = Devonec | first2 = M. | last3 = Perrin | first3 = P. | last4 = Ruffion | first4 = A. | last5 = Decaussin-Petrucci | first5 = M. | last6 = Akin | first6 = O. | last7 = Sheinfeld | first7 = J. | last8 = Guillonneau | first8 = B. | title = Association between sarcoidosis and testicular carcinoma: a diagnostic pitfall. | journal = Sarcoidosis Vasc Diffuse Lung Dis | volume = 24 | issue = 2 | pages = 95-101 | month = Sep | year = 2007 | doi =  | PMID = 18496978 }}</ref> - esp. [[seminoma]].<ref name=pmid17240627>{{Cite journal  | last1 = Jankilevich | first1 = G. | last2 = Mendizabal | first2 = J. | last3 = Massa | first3 = MA. | last4 = Pedernera | first4 = A. | last5 = Galmes | first5 = M. | last6 = Spizzamiglio | first6 = N. | title = [Mediastinal sarcoidal reaction in follow up for seminoma]. | journal = Medicina (B Aires) | volume = 66 | issue = 6 | pages = 552-4 | month =  | year = 2006 | doi =  | PMID = 17240627 }}</ref>
</ref>
**[[Lung adenocarcinoma]].<ref name=pmid22499972>{{Cite journal  | last1 = Tao | first1 = H. | last2 = Yamamoto | first2 = H. | last3 = Matsuda | first3 = E. | last4 = Sano | first4 = F. | last5 = Okabe | first5 = K. | last6 = Sugi | first6 = K. | title = Severe bronchoconstriction due to sarcoid-like reaction to lung cancer. | journal = Asian Cardiovasc Thorac Ann | volume = 20 | issue = 2 | pages = 199-201 | month = Apr | year = 2012 | doi = 10.1177/0218492311431493 | PMID = 22499972 }}</ref>
*[[Seminoma]].<ref name=pmid17240627>{{Cite journal  | last1 = Jankilevich | first1 = G. | last2 = Mendizabal | first2 = J. | last3 = Massa | first3 = MA. | last4 = Pedernera | first4 = A. | last5 = Galmes | first5 = M. | last6 = Spizzamiglio | first6 = N. | title = [Mediastinal sarcoidal reaction in follow up for seminoma]. | journal = Medicina (B Aires) | volume = 66 | issue = 6 | pages = 552-4 | month =  | year = 2006 | doi =  | PMID = 17240627 }}</ref>


===Images===
===Images===
<gallery>
<gallery>
Image:Asteroid_body_intermed_mag.jpg | Sarcoidosis - lung. (WC)
Image:Asteroid_body_intermed_mag.jpg | Sarcoidosis - lung. (WC)
Image:Asteroid_body_very_high_mag.jpg | Granulomata in sarcoidosis with asteroid bodies. (WC)
Image:Asteroid_body_very_high_mag.jpg | Granulomata in sarcoidosis with [[asteroid bodies]]. (WC)
File:Sarcoidosis - Schaumann body (6151514639).jpg | [[Schaumann body]] in sarcoidosis. (WC/Yale Rosen)
File:Sarcoidosis - Fibrosis of granulomas (6148634442).jpg | Fibrosis in sarcoid granulomas. (WC/Yale Rosen)
File:Sarkoidosis muscle.jpg | Muscle involvement in sarcoidosis. (WC/jensflorian)
File:Sarcoidosis_histology_skin_involvement.jpg | Skin involvement in sarcoidosis. (WC/jensflorian)
</gallery>
====Case====
<gallery>
Image: Sarcoidosis - lung FNA -- very low mag.jpg | Sarcoidosis lung - very low mag. (WC)
Image: Sarcoidosis - lung FNA -- low mag.jpg | Sarcoidosis lung - low mag. (WC)
Image: Sarcoidosis - lung FNA -- intermed mag.jpg | Sarcoidosis lung - intermed. mag. (WC)
Image: Sarcoidosis - lung FNA -- high mag.jpg | Sarcoidosis lung - high mag. (WC)
Image: Sarcoidosis - lung FNA -- very high mag.jpg | Sarcoidosis lung - very high mag. (WC)
</gallery>
</gallery>
www:
====www====
*[http://path.upmc.edu/cases/case412.html Sarcoidosis - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case412.html Sarcoidosis - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case517.html Neurosarcoidosis - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case517.html Neurosarcoidosis - several images (upmc.edu)].


==Stains==
==Stains==
*ZN -ve.
*[[AFB stain|AFB]] -ve.
*PASD -ve.
*[[PASD stain|PASD]] -ve.
*GMS -ve.
*[[GMS]] -ve.


Note:
Note:
*Done to r/o infection.
*Must be done to exclude infection.
 
==Molecular==
*PCR for Tuberculosis -ve.


==Sign out==
==Sign out==
*Should be something like ''sarcoid-like granulomas'' and ''clinical correlation required''.
*Should be something like ''sarcoid-like granulomas'' and ''clinical correlation required''.
<pre>
A. RIGHT LOWER LOBE LUNG, WEDGE RESECTION:
- GRANULOMATOUS INFLAMMATION, NON-NECROTIZING (SARCOID-LIKE), SEE COMMENT.
B. RIGHT MIDDLE LOBE LUNG, WEDGE RESECTION:
- GRANULOMATOUS INFLAMMATION, NON-NECROTIZING (SARCOID-LIKE), SEE COMMENT.
C. RIGHT LOWER LOBE LUNG, WEDGE RESECTION:
- GRANULOMATOUS INFLAMMATION, NON-NECROTIZING (SARCOID-LIKE), SEE COMMENT.
COMMENT:
The sections show multiple sarcoid-like granulomas with both centrilobular and
septal involvement. There is a slight upper lobe predominance of the disease.
The main histomorphologic differential diagnoses are: sarcoidosis,
infectious granulomatous pneumonia.
Sarcoidosis is favoured based on the morphology of the granulomas and the
lack of microorganisms with special stains (ZN, PASF, GMS).
A serum ACE level should be considered, if not already done.
Clinical and radiologic correlation is required.
</pre>


==See also==
==See also==
*[[Medical lung diseases]].
*[[Medical lung diseases]].
*[[Cardiac sarcoidosis]].
*[[Cardiac sarcoidosis]].
*[[Melkersson-Rosenthal syndrome]].


==References==
==References==
Michael
48,466

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