Hepatic Sarcoidosis: Natural History and Management Implications.

Mai Sedki,Nicholas Fonseca,Liege Diaz,Cynthia Levy,Priscila Santiago,Mehdi Mirsaeidi,Monica Garcia-Buitrago

doi:10.3389/fmed.2019.00232

Abstract

Introduction: Hepatic granulomas are common in patients with sarcoidosis, but clinically significant liver disease is uncommon and poorly studied. We aimed to characterize the frequency and clinical course of hepatic sarcoidosis in an ethnically diverse population.Methods: This is a retrospective study including all cases of hepatic sarcoidosis in a single center. The median follow-up time was 49 months (4–121). Cases were identified based on ICD-9 and ICD-10 codes for granulomatous hepatitis, sarcoidosis, and hepatic sarcoidosis. The Chi-square and Wilcoxon-signed rank tests were used as indicated to assess for differences between groups.Results: Of 286 patients with sarcoidosis, 27 had hepatic involvement; 78% were female and 48% African American. The most common pattern of liver tests abnormalities was cholestatic. Ten patients had clinically significant hepatic involvement: cirrhosis in seven (25.9%), portal hypertension in nine (33%), and portal vein thrombosis in one (3.7%). Sex, race, and ethnicity were not associated with an increased risk of hepatic involvement or symptomatic hepatic sarcoidosis. Most patients received medical treatment, most commonly oral glucocorticoids. At the end of the follow-up period, all patients were alive but two had undergone liver transplantation due to complications of hepatic sarcoidosis. Three patients with hepatic sarcoidosis had initially been classified as AMA-negative PBC.Conclusions: Hepatic sarcoidosis was found in 9.4% of patients with sarcoidosis and was clinically significant in 37% of those. Identifying and monitoring hepatic sarcoidosis is crucial given its potential complications.

Highlights

Hepatic granulomas are common in patients with sarcoidosis, but clinically significant liver disease is uncommon and poorly studied
Patients with anti-mitochondrial antibody (AMA)-positive primary biliary cholangitis (PBC), idiopathic granulomatous hepatitis, tuberculosis and those with insufficient/incorrect diagnosis were excluded from further analyses (n = 326)
27 had hepatic involvement: 18 with a definitive diagnosis based on biopsy findings and nine with a suspected diagnosis: six were diagnosed based on medical history and three were initially misdiagnosed with AMA-negative PBC which was later modified to hepatic sarcoidosis

Summary

Introduction

Hepatic granulomas are common in patients with sarcoidosis, but clinically significant liver disease is uncommon and poorly studied. Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized by the presence of non-caseating granulomas in affected organs [1]. Hepatic granulomas can be found in 50–65% of patients with sarcoidosis, the clinical consequences of hepatic involvement are variable and symptomatic disease occurs in only 5–15% of patients [2,3,4]. The non-caseating granulomas of hepatic sarcoidosis are often located along the portal tract, several other conditions can cause granulomatous lesions in the liver. Diagnosis of hepatic sarcoidosis is generally based upon the presence of hepatic granulomas, evidence of multiorgan involvement, negative staining and culture for acid-fast bacilli and other bacterial and fungal infections, and exclusion of liver malignancy and drug-induced granulomas [9]

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