Hepatic sarcoidosis: a case series

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.

Clinical Characteristics and Outcome of Hepatic Sarcoidosis: A Population-Based Study 1976–2013

“…Hyperbilirubinemia was uncommon and liver synthetic function was preserved in all patients. These observations are in line with data from previous cohort (16, 18–20). ACE was elevated in only 60% of tested patients which suggest its poor sensitivity for diagnosis of hepatic sarcoidosis, similar to its poor performance for diagnosis of systemic sarcoidosis (21, 22).…”

“…On the other hand, liver biopsy findings of non-caseating granuloma, detected in 14 out of 16 biopsies (88%) have higher sensitivity for the diagnosis of hepatic sarcoidosis. The high sensitivity of liver biopsy has been consistently demonstrated in several cohorts (15, 16, 18). Therefore, negative imaging study does not exclude hepatic sarcoidosis, and liver biopsy should be performed if the clinical suspicion remains high.…”

Particularities of Hepatic Sarcoidosis

“…A recent case series of 7 patients with liver sarcoidosis found that the liver was affected in the evolution of known sarcoidosis in 2 cases, while the other 5 had liver involvement at the time of diagnosis [26]. Four patients presented with upper right quadrant abdominal pain and one patient had incidentally discovered altered liver enzymes on routine evaluation.…”

Extrapulmonary sarcoidosis primarily presenting as cholestatic liver disease

“…Out of 289 records, 33 articles were retrieved. These included 9 review articles,8–16 2 cohort studies,17 18 and 22 case reports or series, including a total of 96 cases 19–40…”

“…Interestingly, in most of the reported cases, biochemical abnormalities were cholestatic rather than hepatocellular 17 22 27. Ursodeoxycholic acid (UDCA) is an agent used to treat cholestasis.…”