1. Extrapulmonary sarcoidosis primarily presenting as cholestatic liver disease
- Author
-
An Vonck, Anne Hoorens, Pieter De Mulder, and Bert Maertens
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Sarcoidosis ,Inflammation ,Cholestasis, Intrahepatic ,Diagnosis, Differential ,03 medical and health sciences ,Liver disease ,0302 clinical medicine ,Cholestasis ,Adrenal Cortex Hormones ,Rare Disease ,medicine ,Humans ,030212 general & internal medicine ,medicine.diagnostic_test ,business.industry ,Liver Diseases ,technology, industry, and agriculture ,General Medicine ,respiratory system ,Middle Aged ,medicine.disease ,Ursodeoxycholic acid ,Granulomatous Hepatitis ,030211 gastroenterology & hepatology ,Cholestatic liver disease ,medicine.symptom ,business ,Liver function tests ,medicine.drug - 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.
- Published
- 2019