S5680 Esophageal Variceal Bleeding: An Uncommon Manifestation of Hepatic Sarcoidosis-Induced Portal Hypertension

Introduction: Sarcoidosis is a multisystem granulomatous disorder that affects the lungs most commonly, however liver, skin, and cardiac manifestations are well described. Manifestations of hepatic involvement range from asymptomatic with only biochemical abnormalities to severe including portal hypertension with or without cirrhosis. Here, we present a case of hepatic sarcoidosis manifesting as non-cirrhotic portal hypertension with variceal bleeding. Case Description/Methods: Patient is a 59-year-old man with Graves’ disease, pulmonary sarcoidosis (off all medication), hypertension, and chronic kidney disease, stage 3, who presented with hematemesis. Importantly, he also has a history of unexplored cholestatic liver enzyme elevation. Notable labs on admission included hemoglobin of 9 g/dL (baseline 11-12 g/dL), creatinine 1.94 mg/dL (baseline 1.5 mg/dL), and aspartate aminotransferase/alanine aminotransferase/alkaline phosphatase U/L were 75/101/422 U/L respectively. An emergent esophagogastroduodenoscopy revealed large (>5 mm) esophageal varices, of which 4 were banded. Relevant infectious/serologic workup include an unremarkable hepatitis panel, alcohol level, ferritin, ceruloplasmin, and antimitochondrial antibody. Antinuclear antibody (1:640) and anti-smooth muscle antibody (1:80) were both positive. MRCP showed diffusely heterogenous enhancement of the liver parenchyma with central peribiliary wall thickening and enhancement. He underwent a percutaneous liver biopsy, with pathology demonstrating non-necrotizing granulomas with surrounding chronic inflammation and fibrosis. The pattern of infiltrate is compatible with hepatic sarcoidosis in this clinical context. Notably, there was no cirrhosis identified on the biopsy. A multidisciplinary discussion is underway between GI and pulmonology regarding treatment, preliminarily favoring initiation of mycophenolate. Discussion: We highlight the importance of maintaining a broad differential for portal hypertension, including non-cirrhotic etiologies such as hepatic sarcoidosis. Symptomatic hepatic sarcoid typically presents with abdominal pain, pruritus, and hepatomegaly, with portal hypertension serving as a much less common manifestation. Liver biopsy is recommended for those with moderate or severe liver enzyme abnormalities or when diagnostic confirmation is needed. Timely identification of hepatic involvement is key to prevent potential disease progression. Some experts propose that patients with symptomatic hepatic sarcoidosis receive prednisone, however further studies are warranted to determine an optimal treatment regimen for hepatic sarcoidosis.