American Journal of Medical Case Reports
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American Journal of Medical Case Reports. 2020, 8(7), 210-215
DOI: 10.12691/ajmcr-8-7-13
Open AccessCase Report

Extrapulmonary Involvement in Sarcoidosis: A Case Report

Courtney Connelly1, Abida Hasan1, Zachary Chung1, Erli Mingomataj1, Vinodkumar Velayudhan1 and Isabel M. McFarlane1,

1Department of Internal Medicine, State University of New York- Downstate Health Science University, Brooklyn, NY, USA- 11203

Pub. Date: April 25, 2020

Cite this paper:
Courtney Connelly, Abida Hasan, Zachary Chung, Erli Mingomataj, Vinodkumar Velayudhan and Isabel M. McFarlane. Extrapulmonary Involvement in Sarcoidosis: A Case Report. American Journal of Medical Case Reports. 2020; 8(7):210-215. doi: 10.12691/ajmcr-8-7-13

Abstract

Sarcoidosis is an idiopathic multisystem granulomatous disease that affects patients of all races and ethnic groups however predilection for women and African Americans is apparent. Extrapulmonary manifestations of sarcoidosis occur in up to 50% of cases. The most common sites of extrapulmonary involvement are the skin, eyes, liver, and reticuloendothelial system followed bt renal, cardiac, and neurological involvement. We present the case of a middle age man with exclusive extrapulmonary sarcoidosis affecting the renal, cardiac, hepatic, splenic and central nervous system. The patient sustained a second episode of venous thromboembolism which highlights a frequently seen complication of sarcoidosis. We discuss the proposed pathophysiology for the prothrombotic state seen in sarcoidosis and imaging modalities that can be utilized to assess extrapulmonary involvement in sarcoidosis. Lastly, sarcoidosis management is reviewed, highlighting that immunosuppressants and tumor necrosis factor inhibitors are being proposed to arrest disease progression and reduce glucocorticoid doses.

Keywords:
sarcoidosis extrapulmonary manifestations cardiac sarcoidosis hepatic sarcoidosis neuro-sarcoidosis renal sarcoidosis venous thromboembolism arterial aneurysm stroke end stage renal disease

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References:

[1]  Iannuzzi, M.C., B.A. Rybicki, and A.S. Teirstein, Sarcoidosis. N Engl J Med, 2007. 357(21): p. 2153-65.
 
[2]  Rybicki, B.A., et al., Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol, 1997. 145(3): p. 234-41.
 
[3]  Ungprasert, P., C.S. Crowson, and E.L. Matteson, Epidemiology and clinical characteristics of sarcoidosis: an update from a population-based cohort study from Olmsted County, Minnesota. Reumatismo, 2017. 69(1): p. 16-22.
 
[4]  Arkema, E.V. and Y.C. Cozier, Epidemiology of sarcoidosis: current findings and future directions. Ther Adv Chronic Dis, 2018. 9(11): p. 227-240.
 
[5]  Baughman, R.P., et al., Sarcoidosis in America. Analysis Based on Health Care Use. Ann Am Thorac Soc, 2016. 13(8): p. 1244-52.
 
[6]  Carmona, E.M., S. Kalra, and J.H. Ryu, Pulmonary Sarcoidosis: Diagnosis and Treatment. Mayo Clin Proc, 2016. 91(7): p. 946-54.
 
[7]  Gerke, A.K., Morbidity and mortality in sarcoidosis. Curr Opin Pulm Med, 2014. 20(5): p. 472-8.
 
[8]  James, W.E., et al., Clinical Features of Extrapulmonary Sarcoidosis Without Lung Involvement. Chest, 2018. 154(2): p. 349-356.
 
[9]  Soto-Gomez, N., J.I. Peters, and A.M. Nambiar, Diagnosis and Management of Sarcoidosis. Am Fam Physician, 2016. 93(10): p. 840-8.
 
[10]  Terushkin, V., et al., Neurosarcoidosis: presentations and management. Neurologist, 2010. 16(1): p. 2-15.
 
[11]  Ungprasert, P., C.S. Crowson, and E.L. Matteson, Association of Sarcoidosis With Increased Risk of VTE: A Population-Based Study, 1976 to 2013. Chest, 2017. 151(2): p. 425-430.
 
[12]  Ungprasert, P., et al., Sarcoidosis and risk of venous thromboembolism: A systematic review and meta-analysis. Sarcoidosis Vasc Diffuse Lung Dis, 2015. 32(3): p. 182-7.
 
[13]  Yaqoob, Z.J., S.G. Al-Kindi, and J.G. Zein, Sarcoidosis and Risk of VTE: Validation With Big Data. Chest, 2017. 151(6): p. 1398-1399.
 
[14]  Lee, J.J. and J.E. Pope, A meta-analysis of the risk of venous thromboembolism in inflammatory rheumatic diseases. Arthritis Res Ther, 2014. 16(5): p. 435.
 
[15]  Ramagopalan, S.V., et al., Risk of venous thromboembolism in people admitted to hospital with selected immune-mediated diseases: record-linkage study. BMC Med, 2011. 9: p. 1.
 
[16]  Judson, M.A., et al., Two year prognosis of sarcoidosis: the ACCESS experience. Sarcoidosis Vasc Diffuse Lung Dis, 2003. 20(3): p. 204-11.
 
[17]  Johns, C.J., et al., Longitudinal study of chronic sarcoidosis with low-dose maintenance corticosteroid therapy. Outcome and complications. Ann N Y Acad Sci, 1986. 465: p. 702-12.
 
[18]  Birnie, D.H., et al., HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm, 2014. 11(7): p. 1305-23.
 
[19]  Ipek, E., et al., Sarcoidosis and the heart: A review of the literature. Intractable Rare Dis Res, 2015. 4(4): p. 170-80.
 
[20]  Nagai, T., et al., Incidence and prognostic significance of myocardial late gadolinium enhancement in patients with sarcoidosis without cardiac manifestation. Chest, 2014. 146(4): p. 1064-1072.
 
[21]  Mehta, D., et al., Cardiac involvement in patients with sarcoidosis: diagnostic and prognostic value of outpatient testing. Chest, 2008. 133(6): p. 1426-1435.
 
[22]  Nery, P.B., et al., Prevalence of cardiac sarcoidosis in patients presenting with monomorphic ventricular tachycardia. Pacing Clin Electrophysiol, 2014. 37(3): p. 364-74.
 
[23]  Cummings, K.W., et al., A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR imaging. Radiographics, 2009. 29(1): p. 89-103.
 
[24]  Ichinose, A., et al., MRI of cardiac sarcoidosis: basal and subepicardial localization of myocardial lesions and their effect on left ventricular function. AJR Am J Roentgenol, 2008. 191(3): p. 862-9.
 
[25]  Vogel-Claussen, J., et al., Delayed enhancement MR imaging: utility in myocardial assessment. Radiographics, 2006. 26(3): p. 795-810.
 
[26]  Chareonthaitawee, P., et al., Joint SNMMI-ASNC Expert Consensus Document on the Role of (18)F-FDG PET/CT in Cardiac Sarcoid Detection and Therapy Monitoring. J Nucl Med, 2017. 58(8): p. 1341-1353.
 
[27]  Ganeshan, D., et al., Sarcoidosis from Head to Toe: What the Radiologist Needs to Know. Radiographics, 2018. 38(4): p. 1180-1200.
 
[28]  Blankstein, R., et al., Cardiac positron emission tomography enhances prognostic assessments of patients with suspected cardiac sarcoidosis. J Am Coll Cardiol, 2014. 63(4): p. 329-36.
 
[29]  Mc Ardle, B.A., et al., The role of F(18)-fluorodeoxyglucose positron emission tomography in guiding diagnosis and management in patients with known or suspected cardiac sarcoidosis. J Nucl Cardiol, 2013. 20(2): p. 297-306.
 
[30]  Iwai, K., et al., Pathological studies on sarcoidosis autopsy. I. Epidemiological features of 320 cases in Japan. Acta Pathol Jpn, 1993. 43(7-8): p. 372-6.
 
[31]  Shah, R., G.H. Roberson, and J.K. Cure, Correlation of MR imaging findings and clinical manifestations in neurosarcoidosis. AJNR Am J Neuroradiol, 2009. 30(5): p. 953-61.
 
[32]  Smith, J.K., M.G. Matheus, and M. Castillo, Imaging manifestations of neurosarcoidosis. AJR Am J Roentgenol, 2004. 182(2): p. 289-95.
 
[33]  Zajicek, J.P., et al., Central nervous system sarcoidosis--diagnosis and management. QJM, 1999. 92(2): p. 103-17.
 
[34]  Koyama, T., et al., Radiologic manifestations of sarcoidosis in various organs. Radiographics, 2004. 24(1): p. 87-104.
 
[35]  Warshauer, D.M. and J.K. Lee, Imaging manifestations of abdominal sarcoidosis. AJR Am J Roentgenol, 2004. 182(1): p. 15-28.
 
[36]  Britt, A.R., et al., Sarcoidosis: abdominal manifestations at CT. Radiology, 1991. 178(1): p. 91-4.
 
[37]  Kessler A, M.D.G., Israel H.L., Goldberg B.B., Hepatic and Splenic Sarcoidosis: Ultrasound and MR Imaging. Abdom Imaging, 1993. 18(2): p. 159-163.
 
[38]  Folz, S.J., C.D. Johnson, and S.J. Swensen, Abdominal manifestations of sarcoidosis in CT studies. J Comput Assist Tomogr, 1995. 19(4): p. 573-9.
 
[39]  Kim, J.S., et al., Cardiac sarcoidosis. Am Heart J, 2009. 157(1): p. 9-21.
 
[40]  Judson, M.A., Advances in the diagnosis and treatment of sarcoidosis. F1000Prime Rep, 2014. 6: p. 89.
 
[41]  Prasse, A., The Diagnosis, Differential Diagnosis, and Treatment of Sarcoidosis. Dtsch Arztebl Int, 2016. 113(33-34): p. 565-74.
 
[42]  Jamilloux, Y., et al., Efficacy and safety of tumor necrosis factor antagonists in refractory sarcoidosis: A multicenter study of 132 patients. Semin Arthritis Rheum, 2017. 47(2): p. 288-294.