P3978 - Calling the Uncommon Common? Hepatic Sarcoidosis Presenting as Cirrhosis and Portal Hypertension

Devanshi Kajal, MD¹, Saeed Graham, MD², Mary-Jane Williams, MD^2
1East Carolina University, Greenville, NC; ²East Carolina University Brody School of Medicine, Greenville, NC

Introduction: Hepatic sarcoidosis is an extrapulmonary manifestation of sarcoidosis with some studies reporting an incidence of 5-25%, but only a few patients (< 1%) go on to develop cirrhosis and portal hypertension as a complication. Diagnosis is often delayed as no single laboratory or imaging finding is confirmatory. Tissue biopsy is necessary. Most patients are asymptomatic, compounding the diagnostic challenge. The following case details a patient who was found to have radiographic evidence of cirrhosis as the first presentation of hepatic sarcoidosis.

Case Description/Methods: A 45 y old African American female presented to the primary care office with fever, night sweats and weight loss. Initial work up showed elevated alkaline phosphatase and computed tomography of the chest and abdomen showed pulmonary nodules, hepatosplenomegaly and diffusely heterogeneous enhancement of the spleen and irregular capsular surface of the liver concerning for cirrhosis and portal hypertension. Further labs revealed negative autoimmune panel, QuantiFERON and very high ACE levels. Meanwhile, she was also admitted to the hospital for severe hypercalcemia. Labs revealed lymphopenia, anemia. At this point, a strong suspicion for multiorgan sarcoidosis with predominant extrapulmonary involvement was raised and a tissue diagnosis was sought for. Liver biopsy was done which revealed mild portal and interface activity with focal plasma rich component, periportal fibrosis with focal bridging and non-necrotizing epithelioid granulomas consistent with sarcoidosis. There was also mild macrovesicular steatosis with mild lobular activity. Of note, the biopsy specimen was noted to be small with only six portal tracts limiting evaluation for cirrhosis however, multiple portal tracts showed relatively large well-formed non-necrotizing epithelioid granulomas. After the tissue diagnosis was obtained, she was lost to follow up for a year but then presented to the hospital twice with persistent hypercalcemia. She was referred to a hepatologist after discharge for additional recommendations. She is currently asymptomatic and is being kept on active surveillance with 6-monthly upper GI endoscopy and right upper quadrant ultrasound as part of cirrhosis management.

Discussion: Cirrhosis and portal hypertension are a rare complication of hepatic sarcoidosis. Even rarer is cirrhosis detected on initial imaging done as a part of diagnostic workup. Early detection and follow up is necessary to avoid complications like overt liver failure.



Disclosures:


Devanshi Kajal, MD¹, Saeed Graham, MD², Mary-Jane Williams, MD². P3978 - Calling the Uncommon Common? Hepatic Sarcoidosis Presenting as Cirrhosis and Portal Hypertension, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.