Sarcoidosis

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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
Diagnosis in short

Sarcoidosis-like granulomas in a lymph node. H&E stain.
LM well-formed granulomas with few surrounding lymphocytes ("naked"), non-necrotizing
LM DDx fungal infections, MAC, tuberculosis, other infections, drug reactions, reactive process to malignancy
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

General

  • Diagnosis of exclusion - infection, neoplasm, 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. ‡
    • Negative for microorganisms with special stains.
  • Usu. minimal (lymphoid) inflammation; sarcoid granulomas are known as "naked granulomas".[8][1]
  • In lung: interstitial location.

Notes:

  • ‡ Reported with necrosis - uncommon.[1]

DDx:

Images

  • Sarcoidosis - lung. (WC)

  • Granulomata in sarcoidosis with asteroid bodies. (WC)

www:

Stains

Note:

  • Done to r/o infection.

Sign out

  • Should be something like sarcoid-like granulomas and clinical correlation required.

See also

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.
  6. ↑ URL: http://www.radiologyassistant.nl/en/46b480a6e4bdc. Accessed on: 23 May 2010.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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.
  12. ↑ 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.
  13. ↑ Paparel, P.; Devonec, M.; Perrin, P.; Ruffion, A.; Decaussin-Petrucci, M.; Akin, O.; Sheinfeld, J.; Guillonneau, B. (Sep 2007). "Association between sarcoidosis and testicular carcinoma: a diagnostic pitfall.". Sarcoidosis Vasc Diffuse Lung Dis 24 (2): 95-101. PMID 18496978.
  14. ↑ 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.
  15. ↑ Tao, H.; Yamamoto, H.; Matsuda, E.; Sano, F.; Okabe, K.; Sugi, K. (Apr 2012). "Severe bronchoconstriction due to sarcoid-like reaction to lung cancer.". Asian Cardiovasc Thorac Ann 20 (2): 199-201. doi:10.1177/0218492311431493. PMID 22499972.
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