Difference between revisions of "Sarcoidosis"
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| Line 14: | Line 14: | ||
| Gross = | | Gross = | ||
| Grossing = | | Grossing = | ||
| Site = [[lung]], hilar lymph nodes of the lung, other sites | | Site = [[lung]], hilar lymph nodes of the lung, [[skin]], [[cardiac sarcoidosis|heart]], other sites | ||
| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Line 33: | Line 33: | ||
*[[Diagnosis of exclusion]] - infection, neoplasms, and drugs must be excluded. | *[[Diagnosis of exclusion]] - infection, neoplasms, and drugs must be excluded. | ||
*Uncommon. | *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: | 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> | *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> | **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== | ||
*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 }} | *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 }} | ||
| Line 42: | Line 44: | ||
*Bilateral hilar lymphadenopathy. | *Bilateral hilar lymphadenopathy. | ||
DDx | 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> | *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> | ||
| Line 48: | Line 50: | ||
==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 ([[PAS-D]], [[GMS]], [[AFB]]). | ||
*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. | ||
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: | ||
Revision as of 03:22, 26 November 2013
| Sarcoidosis | |
|---|---|
| Diagnosis in short | |
_lymph_node_biopsy.jpg) Sarcoidosis-like granulomas in a lymph node. H&E stain. | |
| LM DDx | fungal infections, MAC, tuberculosis, other infections, drug reactions |
| Stains | AFB -ve, GMS -ve, PASD -ve |
| Site | lung, hilar lymph nodes of the lung, skin, heart, other sites |
|
| |
| Prevalence | uncommon |
| Blood work | +/-ACE elevated |
| Radiology | +/-bilateral hilar lymphadenopathy (very common), +/-interstitial pattern, +/- pulmonary infiltrates, +/-cystic/bullous changes |
| Clin. DDx | 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.
General
- Diagnosis of exclusion - infection, neoplasms, and drugs must be excluded.
- Uncommon.
- Afflicits skin ~25% of the time.[1]
Serology:
- Angiotensin-converting enzyme (ACE) - used for diagnosis and to monitor activity.[2][3]
- Elevated in approximately 65% of patients in one series.[4]
Gross
- Lungs - classic location.[5]
- Bilateral hilar lymphadenopathy.
DDx lungs - radiologic:
- Carcinomatosis - interstitial pattern.[6]
- Lymphoma - bilateral lymphadenopathy +/- mediastinal lymphadenopathy.[7]
Microscopic
Features:
- Granulomata, well-formed, non-necrotizing. ‡
- Usu. minimal (lymphoid) inflammation; sarcoid granulomas are known as "naked granulomas".[8][1]
- In lung: interstitial location.
Notes:
- ‡ Reported with necrosis - uncommon.[1]
DDx:
- Reactive changes - may mimic sarcoidosis.
- Drug reaction.
- Infections.
- Common variable immunodeficiency.[12]
- Seminoma.[13]
Images
Sarcoidosis - lung. (WC)
Granulomata in sarcoidosis with asteroid bodies. (WC)
www:
Stains
- ZN -ve.
- PASD -ve.
- GMS -ve.
Note:
- Done to r/o infection.
Sign out
- Should be something like sarcoid-like granulomas and clinical correlation required.
See also
References
- ↑ 1.0 1.1 1.2 Noiles, K.; Beleznay, K.; Crawford, RI.; Au, S.. "Sarcoidosis can present with necrotizing granulomas histologically: two cases of ulcerated sarcoidosis and review of the literature.". J Cutan Med Surg 17 (6): 377-83. PMID 24138972.
- ↑ Kaura, V.; Kaura, NV.; Kaura, BN.; Kaura, CS.. "Angiotensin-converting enzyme inhibitors in the treatment of sarcoidosis and association with ACE gene polymorphism: case series.". Indian J Chest Dis Allied Sci 55 (2): 105-7. PMID 24047001.
- ↑ Stouten, K.; Werken, MV.; Tchetverikov, I.; Saboerali, M.; Vermeer, HJ.; Castel, R.; Verheijen, FM. (Jul 2013). "Extreme elevation of serum angiotensin-converting enzyme (ACE) activity: always consider familial ACE hyperactivity.". Ann Clin Biochem. doi:10.1177/0004563213489812. PMID 23897103.
- ↑ Shorr, AF.; Torrington, KG.; Parker, JM. (Aug 1997). "Serum angiotensin converting enzyme does not correlate with radiographic stage at initial diagnosis of sarcoidosis.". Respir Med 91 (7): 399-401. PMID 9327039.
- ↑ Rao, DA.; Dellaripa, PF. (May 2013). "Extrapulmonary manifestations of sarcoidosis.". Rheum Dis Clin North Am 39 (2): 277-97. doi:10.1016/j.rdc.2013.02.007. PMID 23597964.
- ↑ URL: http://www.radiologyassistant.nl/en/46b480a6e4bdc. Accessed on: 23 May 2010.
- ↑ Boujaoude, Z.; Dahdel, M.; Pratter, M.; Kass, J. (Jan 2012). "Endobronchial ultrasound with transbronchial needle aspiration in the diagnosis of bilateral hilar and mediastinal lymphadenopathy.". J Bronchology Interv Pulmonol 19 (1): 19-23. doi:10.1097/LBR.0b013e3182442b89. PMID 23207258.
- ↑ Brinster, NK. (Nov 2008). "Dermatopathology for the surgical pathologist: a pattern-based approach to the diagnosis of inflammatory skin disorders (part II).". Adv Anat Pathol 15 (6): 350-69. doi:10.1097/PAP.0b013e31818b1ac6. PMID 18948765.
- ↑ Tong, D.; Manolios, N.; Howe, G.; Spencer, D. (Jan 2012). "New onset sarcoid-like granulomatosis developing during anti-TNF therapy: an under-recognised complication.". Intern Med J 42 (1): 89-94. PMID 22389903.
- ↑ Reule, RB.; North, JP. (Nov 2013). "Cutaneous and pulmonary sarcoidosis-like reaction associated with ipilimumab.". J Am Acad Dermatol 69 (5): e272-3. doi:10.1016/j.jaad.2013.07.028. PMID 24124863.
- ↑ Hervier, B.; Wastiaux, H.; Freour, T.; Masseau, A.; Corvec, S.; Armingeat, T.; Hamidou, M. (Sep 2009). "[Sarcoidosis-like granulomatosis revealing a tertiary syphilis].". Rev Med Interne 30 (9): 806-8. doi:10.1016/j.revmed.2009.01.003. PMID 19249139.
- ↑ Vultaggio, A.; Matucci, A.; Parronchi, P.; Rossi, O.; Filì, L.; Giudizi, MG.; Palandri, F.; Agostini, C. et al. (Sep 2007). "Association between sarcoidosis-like disease and common variable immunodeficiency (CVI): a new CVI variant showing an activation of the immune system.". Sarcoidosis Vasc Diffuse Lung Dis 24 (2): 127-33. PMID 18496983.
- ↑ Jankilevich, G.; Mendizabal, J.; Massa, MA.; Pedernera, A.; Galmes, M.; Spizzamiglio, N. (2006). "[Mediastinal sarcoidal reaction in follow up for seminoma].". Medicina (B Aires) 66 (6): 552-4. PMID 17240627.