Extrapulmonary sarcoidosis primarily presenting as cholestatic liver disease

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Abstract

Sarcoidosis is a multisystem inflammatory disorder associated with non-caseating granulomas in affected organs, most commonly the lungs. Involvement of extrapulmonary organs is common, but lack of pulmonary involvement is rare and is called non-pulmonary sarcoidosis (NPS). Like pulmonary sarcoidosis, a definitive diagnostic test for NPS does not exist. Instead, the diagnosis of sarcoidosis requires the following elements: clinical and radiographic manifestations of sarcoidosis, histopathological detection of non-caseating granulomas and the exclusion of other diseases that may present similarly. Because of the experience with corticosteroids in pulmonary sarcoidosis, they are generally considered first-line therapy for NPS too. Ursodeoxycholic acid can be used to reduce cholestasis in NPS, but is inferior to corticosteroids in reducing inflammation. We hereby present a case that is particularly notable for its rare presentation of NPS as a granulomatous hepatitis with cholestatic liver function tests.

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De Mulder, P., Maertens, B., Hoorens, A., & Vonck, A. (2019). Extrapulmonary sarcoidosis primarily presenting as cholestatic liver disease. BMJ Case Reports, 12(12). https://doi.org/10.1136/bcr-2019-232618

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