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Difference between revisions of "Sarcoidosis"
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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 = well-formed [[granuloma]]s with few surrounding lymphocytes ("naked"), non-necrotizing | | Micro = well-formed [[granuloma]]s often with few surrounding lymphocytes ("naked"), usually non-necrotizing | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = fungal infections, [[MAC]], [[tuberculosis]], other infections, drug reactions | | LMDDx = fungal infections, [[MAC]], [[tuberculosis]], other infections, drug reactions, reactive process to malignancy | ||
| Stains = AFB -ve, GMS -ve, PASD -ve | | Stains = AFB -ve, GMS -ve, PASD -ve | ||
| IHC = | | IHC = | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = PCR for [[tuberculosis]] -ve | ||
| IF = | | IF = | ||
| Gross = | | Gross = | ||
| Line 26: | Line 26: | ||
| Prognosis = | | Prognosis = | ||
| Other = | | Other = | ||
| ClinDDx = [[lymphoma]], [[metastatic | | 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 | '''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== | ||
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*Carcinomatosis - interstitial pattern.<ref>URL: [http://www.radiologyassistant.nl/en/46b480a6e4bdc http://www.radiologyassistant.nl/en/46b480a6e4bdc]. Accessed on: 23 May 2010.</ref> | *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> | *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> | ||
<gallery> | |||
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== | ||
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**Negative for microorganisms with special stains. | **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><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> | *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: | *In lung: | ||
**Interstitial location +/- centrilobular. | |||
Notes: | Notes: | ||
| Line 59: | Line 67: | ||
DDx: | DDx: | ||
* | *Infections. | ||
* | **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> | ||
*[[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> | |||
*Reactive changes associated with tumours: | |||
* | **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> | ||
===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)]. | ||
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Note: | Note: | ||
* | *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== | ||