Abstract

Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven.

Highlights

  • Sarcoidosis, a systemic disease of unknown etiology, is characterized by the presence of non caseous granulomas in the affected organs

  • Liver infiltration by granulomas can be responsible for anicteric cholestasis, only a minority of patient progress to portal hypertension, cirrhosis and liver failure

  • The hepatic localization revealed sarcoidosis in 5 cases: 4 patients presented with right upper-quadrant abdominal pain; in 1 case, sarcoidosis was revealed by abnormal liver tests discovered fortuitously trough a regular blood test

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Summary

Introduction

Sarcoidosis, a systemic disease of unknown etiology, is characterized by the presence of non caseous granulomas in the affected organs. It is revealed usually by mediastinal and pulmonary localization, the disease may affect any organ. Liver is a common location, approximately 50-80% of patients with systemic sarcoidosis have hepatic involvement at liver biopsy [1]. It is usually asymptomatic and is manifested by nonspecific biochemical disturbances; it remains undiagnosed in many cases. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement

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