A Case of Severe Cardiac Sarcoidosis with Minimal Pulmonary Involvement

Abstract

Sarcoidosis is a granulomatous disease of unknown etiology. Although sarcoidosis is a systemic disease, there appears to be a predilection for involvement of certain organs. The pulmonary system is the most commonly affected system among all racial groups. Cardiac and respiratory complications are the leading causes of death due to sarcoidosis and in certain patient populations about half of these deaths are attributed to cardiac sarcoidosis. There are few autopsy case reports of cardiac sarcoidosis with minimal respiratory involvement making this case report relevant to the importance of the recognition and awareness of this entity. Acad Forensic Pathol. 2018 8(2): 407-415

Keywords

Forensic pathology Cardiac sarcoidosis Splenic sarcoidosis Noncaseating granuloma Autopsy

Get full access to this article

View all access options for this article.

References

Sharma

. Sarcoidosis: a historical perspective. Clin Dermatol. 2007 5-6; 25(3):232–41. PMID: 17560300. https://doi.org/10.1016/j.clindermatol.2007.03.013.

Roberts

McAllister

Ferrans

. Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (group 1) and review of 78 previously described necropsy patients (group 11). Am J Med. 1977 7; 63(1):86–108. PMID: 327806.

Longcope

Freiman

. A study of sarcoidosis; based on a combined investigation of 160 cases including 30 autopsies from The Johns Hopkins Hospital and Massachusetts General Hospital. Medicine (Baltimore). 1952 2; 31(1):1–132. PMID: 14899212. https://doi.org/10.1097/00005792-195202000-00001.

Reich

. What is sarcoidosis? Chest. 2003 7; 124(1):367–71. PMID: 12853546. https://doi.org/10.1378/chest.124.1.367.

Rybicki

Major

Popovich

. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol. 1997 2 1; 145(3):234–41. PMID: 9012596. https://doi.org/10.1093/oxfordjournals.aje.a009096.

Iannuzzi

Rybicki

Teirstein

. Sarcoidosis. N Engl J Med. 2007 11 22; 357(21):2153–65. PMID: 18032765. https://doi.org/10.1056/nejmra071714.

Costabel U, Hunninghake GW. ATS/ERS/WASOG statement on sarcoidosis. Sarcoidosis Statement Committee. American Thoracic Society. European Respiratory Society. World Association for Sarcoidosis and Other Granulomatous Disorders. Eur Respir J. 1999 10; 14(4):735–7. PMID: 10573213. https://doi.org/10.1034/j.1399-3003.1999.14d02.x.

Iwai

Tachibana

Takemura

. Pathological studies on sarcoidosis autopsy. I. Epidemiological features of 320 cases in Japan. Acta Pathol Jpn. 1993 7-8; 43(7-8):372–6. PMID: 8372682. https://doi.org/10.1111/j.1440-1827.1993.tb01148.x.

Mehta

Lubitz

Frankel

. Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest. 2008 6; 133(6):1426–1435. PMID: 18339784. https://doi.org/10.1378/chest.07-2784.

10.

Semenzato

. ACCESS: a case control etiologic study of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2005 6; 22(2):83–6. PMID: 16053022.

11.

Sharma

. Sarcoidosis around the world. Clin Chest Med. 2008 9; 29(3):357–63, vii. PMID: 18539231. https://doi.org/10.1016/j.ccm.2008.03.013.

12.

Baughman

Teirstein

Judson

. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med. 2001 11 15; 164(10 Pt 1):1885–9. PMID: 11734441.

13.

Warshauer

. Splenic sarcoidosis. Semin Ultrasound CT MR. 2007 2; 28(1):21–7. PMID: 17366705. https://doi.org/10.1053/j.sult.2006.10.004.

14.

Rosen

. Pathology of sarcoidosis. Semin Respir Crit Care Med. 2007 2; 28(1):36–52. PMID: 17330191. https://doi.org/10.1055/s-2007-970332.

15.

Uemura

Morimoto

Hiramitsu

. Histologic diagnostic rate of cardiac sarcoidosis: evaluation of endomyocardial biopsies. Am Heart J. 1999 8; 138(2 Pt 1):299–302. PMID: 10426842. https://doi.org/10.1016/s0002-8703(99)70115-8.

16.

Butany

Leong

Carmichael

Komeda

. A 30-year analysis of cardiac neoplasms at autopsy. Can J Cardiol. 2005 6; 21(8): 675–80. PMID: 16003450.

17.

Grebenc

Rosado de Christenson

Burke

. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 2000 7-8; 20(4):1073–103; quiz 1110 -1, 1112. PMID: 10903697. https://doi.org/10.1148/radiographics.20.4.g00jl081073.

18.

Burke

Virmani

. Tumors of the heart and great vessels. Fascicle 16, 3 rd Series: Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1996.

19.

Miguel

Bestetti

. Primary cardiac lymphoma. Int J Cardiol. 2011 6 16; 149(3):358–63. PMID: 20227122. https://doi.org/10.1016/j.ijcard.2010.02.016.

20.

Chiles

Woodard

Gutierrez

Link

. Metastatic involvement of the heart and pericardium: CT and MR imaging. Radiographics. 2001 3-4; 21(2):439–49. PMID: 11259706. https://doi.org/10.1148/radiographics.21.2.g01mr15439.

21.

Hudzik

Miszalski-Jamka

Glowacki

. Malignant tumors of the heart. Cancer Epidemiol. 2015 10; 39(5):665–72. PMID: 26239627. https://doi.org/10.1016/j.canep.2015.07.007.

22.

Goldberg

Blankstein

Padera

. Tumors metastatic to the heart. Circulation. 2013 10 15; 128(16):1790–4. PMID: 24126323. https://doi.org/10.1161/circulationaha.112.000790.

23.

Ekmektzoglou

Samelis

Xanthos

. Heart and tumors: location, metastasis, clinical manifestations, diagnostic approaches and therapeutic considerations. J Cardiovasc Med (Hagerstown). 2008 8; 9(8):769–77. PMID: 18607239. https://doi.org/10.2459/jcm.0b013e3282f88e49.

24.

McDonnell

Mann

Bulkley

. Involvement of the heart by malignant lymphoma: a clinicopathologic study. Cancer. 1982 3 1; 49(5):944–51. PMID: 7037154. https://doi.org/10.1002/1097-0142(19820301)49:5%3C944::%20aid-cncr2820490519%3E3.0.co;2-c.

25.

James

. A clinicopathological classification of granulomatous dis orders. Postgrad Med J. 2000 8; 76(898):457–65. PMID: 10908370. PMCID: PMC1741697. https://doi.org/10.1136/pmj.76.898.457.

26.

Sakthivel

Bruder

. Mechanism of granuloma formation in sarcoidosis. Curr Opin Hematol. 2017 1; 24(1):59–65. PMID: 27755127. https://doi.org/10.1097/moh.0000000000000301.

27.

Lagana

Parwani

Nichols

. Cardiac sarcoidosis: a pathology-focused review. Arch Pathol Lab Med. 2010 7; 134(7):1039–46. PMID: 20586635. https://doi.org/10.1043/2009-0274-RA.1.

28.

Gal

Koss

. The pathology of sarcoidosis. Curr Opin Pulm Med. 2002 9; 8(5):445–51. PMID: 12172451. https://doi.org/10.1097/00063198-200209000-00018.

29.

Okura

Dec

Hare

. A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis. J Am Coll Cardiol. 2003 1 1; 41(2):322–9. PMID: 12535829. https://doi.org/10.1016/s0735-1097(02)02715-8.

30.

Agostini

Adami

Semenzato

. New pathogenetic insights into the sarcoid granuloma. Curr Opin Rheumatol. 2000 1; 12(1):71–6. PMID: 10647958. https://doi.org/10.1097/00002281-200001000-00012.

31.

Lazarus

. Sarcoidosis: epidemiology, etiology, pathogenesis, and genetics. Dis Mon. 2009 11; 55(11):649–60. PMID: 19857640. https://doi.org/10.1016/j.disamonth.2009.04.008.

32.

Gal

Koss

. The pathology of pulmonary sarcoidosis: update. Semin Diagn Pathol. 2007 8; 24(3):150–61. PMID: 17882899. https://doi.org/10.1053/j.semdp.2007.06.002.

33.

Gerke

Hunninghake

. The immunology of sarcoidosis. Clin Chest Med. 2008 9; 29(3):379–90, vii. PMID: 18539233. https://doi.org/10.1016/j.ccm.2008.03.014.

34.

Schoppet

Pankuweit

Maisch

. Cardiac sarcoidosis: cytokine patterns in the course of the disease. Arch Pathol Lab Med. 2003 9; 127(9):1207–10. PMID: 12946220. https://doi.org/10.1043/1543-2165(2003)127<1207:%20CSCPIT>2.0.CO;2.

35.

Wahlström

Katchar

Wigzell

. Analysis of intracellular cytokines in CD4+ and CD8+ lung and blood T cells in sarcoidosis. Am J Respir Crit Care Med. 2001 1; 163(1):115–21. PMID: 11208635. https://doi.org/10.1164/ajrccm.163.1.9906071.

36.

Smedema

Snoep

van Kroonenburgh

. Cardiac involvement in patients with pulmonary sarcoidosis assessed at two university medical centers in the Netherlands. Chest. 2005 7; 128(1): 30–5. PMID: 16002912. https://doi.org/10.1378/chest.128.1.30.

37.

Yeboah

Lee

Sharma

. Cardiac sarcoidosis: a review 2011. Curr Opin Pulm Med. 2011 9; 17(5):308–15. PMID: 21743332. https://doi.org/10.1097/mcp.0b013e328349637a.

38.

Kim

Judson

Donnino

. Cardiac sarcoidosis. Am Heart J. 2009 1; 157(1):9–21. PMID: 19081391. https://doi.org/10.1016/j.ahj.2008.09.009.

39.

Kandolin

Lehtonen

Airaksinen

. Cardiac sarcoidosis: epidemiology, characteristics, and outcome over 25 years in a nationwide study. Circulation. 2015 2 17; 131(7):624–32. PMID: 25527698. https://doi.org/10.1161/circulationaha.114.011522.

40.

Yotsumoto

. Fever, hypercalcemia and tuberculosis. Intern Med. 2007; 46(6):259–60. PMID: 17379990. https://doi.org/10.2169/internalmedicine.46.0176.