Hepatic sarcoidosis

Sarcoidosis is a systemic disease of unknown etiology involving multiple organs. Main manifestations are biliary lymphadenopathy, pulmonary densification as well as skin, joint and eye involvement. However, liver involvement was estimated to occur in up to 60 % of all patient. As many patients experience only minor symptoms, a high number of undiagnosed cases must be assumed. In order to identify patients with hepatic sarcoidosis a throughout characterization of these patients and their course of disease is necessary. However, the currently available evidence on hepatic sarcoidosis were collected from the 1970s when treatment of liver disease was clearly different form today”s standards. Thus, we collected 43 patients from 4 German centers to evaluate current treatment standards and course of disease. All our patients had liver biopsy with histologically proven granulomatous hepatitis to confirm sarcoidosis. Detailed characterization of these patients showed an overall benign course of disease. Treatment of these patients was very diverse with glucocorticoids for 1 year in 57 %, 5-10 years in 17 %, and permanently in 20 % of patients. Other treatments included DMARD in 23 % of patients (40 % azathioprine, 23 % MTX, 5 % hydroxychloroquine, 5 % MMF, and 5 % cyclophosphamide). Biologicals were administered to 7 % of all patients. Despite these very diverse treatments patients generally showed only slow progress. None of our patients received liver transplantation, or suffered from significant liver related morbidity, or mortality.

Overall, our cohort indicated that morbidity and mortality of sarcoidosis as a systemic disease is mostly not dependent on hepatic manifestation as progression is slow and symptoms generally mild.

Publikationsverlauf

Artikel online veröffentlicht:
04. Januar 2021