Sarcoidosis

Sarcoidosis
	Diagnosis in short
	; 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, 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.

Serology:

Angiotensin-converting enzyme (ACE) - used for diagnosis and to monitor activity.^[1]^[2]
- Elevated in approximately 65% of patients in one series.^[3]

Gross

Lungs - classic location.^[4]
Bilateral hilar lymphadenopathy.

DDx (radiologic):

Carcinomatosis - interstitial pattern.^[5]
Lymphoma - bilateral lymphadenopathy +/- mediastinal lymphadenopathy.^[6]

Microscopic

Features:

Granulomata, well-formed, non-necrotizing.
- Negative for microorganisms with special stains (PAS-D, GMS, AFB).
Usu. minimal (lymphoid) inflammation; sarcoid granulomas are known as "naked granulomas".^[7]
In lung: interstitial location.

DDx:

Reactive changes - may mimic sarcoidosis.
Drug reaction.
- Anti-TNF therapy.^[8]
- Ipilimumab.^[9]
Infections.
- Syphilis.^[10]
Common variable immunodeficiency.^[11]
Seminoma.^[12]

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.

References

↑ 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.

[pmid24047001-1] 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.

[pmid23897103-2] 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.

[pmid9327039-3] 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.

[pmid23597964-4] 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.

[5] URL: http://www.radiologyassistant.nl/en/46b480a6e4bdc. Accessed on: 23 May 2010.

[pmid23207258-6] 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.

[pmid18948765-7] 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.

[pmid22389903-8] 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.

[pmid24124863-9] 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.

[pmid19249139-10] 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.

[pmid18496983-11] 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.

[pmid17240627-12] 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.

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Sarcoidosis

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