Extrapulmonary sarcoidosis primarily presenting as cholestatic liver disease

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.

Hepatic sarcoidosis resembling primary sclerosing cholangitis

“…Intrahepatic sarcoidosis can resemble primary sclerosing cholangitis (PSC) or in rare circumstances these two entities can coexist. 8–12 …”

“…Intrahepatic sarcoidosis can resemble primary sclerosing cholangitis (PSC) or in rare circumstances these two entities can coexist. [8][9][10][11][12] We present a case report of a patient with radiological findings 'typical' for PSC and liver biopsy showed non-caseating granulomas consistent with hepatic sarcoidosis.…”

The clinical management of hepatic sarcoidosis: A systematic review

“… 9 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 The dosage of corticosteroids, particularly prednisolone, varies from 20 mg to 60 mg per day. 12 , 27 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 In our review, some of the studies used dosages of 0.5 mg to 1 mg/kg/day, 45 , 46 , 47 and none of the studies reported a prednisolone dose of greater than 1 mg/kg/day. Two review articles suggested a time frame of 4–6 weeks for initial duration of treatment, and subsequently to taper off prednisolone 5‐10 mg every 4–8 weeks.…”

“…The indication for therapy has been based on the presence of symptoms and biochemical liver derangement, with or without evidence of fibrosis/ cirrhosis on imaging 9,27–36 . The dosage of corticosteroids, particularly prednisolone, varies from 20 mg to 60 mg per day 12,27,37–44 . In our review, some of the studies used dosages of 0.5 mg to 1 mg/kg/day, 45–47 and none of the studies reported a prednisolone dose of greater than 1 mg/kg/day.…”