A Rare Case of Isolated Hepatic Sarcoidosis With Cholestatic Jaundice and Review of Literature

Abstract

Introduction: Sarcoidosis is a chronic, granulomatous multisystemic disease of unknown etiology. It can involve any organ, but mostly affects lung, lymph nodes, eyes, and skin. The prevalence is about 1-40/100,000, and isolated liver involvement is only about 13%, which is twice as common in African Americans. Cholestatic jaundice is rare, and seen in less than 5% of cases. We are reporting one such patient. A 31-year-old African American male with history of G6PD deficiency presented with rightsided abdominal pain, fever of 102 F, weight loss, pruritus, and jaundice. On exam, he was icteric and tachycardic with RUQ tenderness. He had hepatomegaly, but no signs of cirrhosis. He had normal CBC, ESR 57, INR 1, protein 9, albumin 2.7, bilirubin 8.8 with direct 7, AST 318, ALT 370, and alkaline phosphatase 1,207. Hepatitis panel for A, B, and C viruses, iron saturation, AMA, ASMA, and SLA antibodies were negative; ANA was positive with 1:40 titer. He had history of hepatic sarcoidosis for which he was on steroids in the past. Steroid was stopped and transitioned to azathioprine due to avascular necrosis of the hip, which normalized his LFTs, but he was lost to follow-up. CXR was negative for pulmonary sarcoidosis. MRCP did not reveal strictures in CBD or extrinsic compression to explain high alkaline phosphatase, but showed multiple nodular densities in the liver. Repeat liver biopsy showed multiple non-caseating epithelioid granulomata consistent with sarcoidosis. He was started on UDCA 300 mg orally twice a day and budesonide 9 mg daily with plan to transition to Azathioprine. Hepatic sarcoidosis is mostly asymptomatic. Hepatomegaly can be seen in only 5-10%, but histologically, it is found in 50-80% with systemic sarcoidosis. Few patients progress to severe cholestatic jaundice, PHTN, Budd-Chiari syndrome, and cirrhosis. Chronic intrahepatic cholestasis is common in African Americans due to progressive destruction of bile ducts by portal and periportal granulomas. Alkaline phosphatase level is usually 5-10 times the normal limit or more. Hepatomegaly can occur with discrete nodules, which can become confluent and mistaken for HCC. The most important differential diagnosis is PBC, where the granulomas are few and poorly differentiated, and hepatitis C, where they have a predilection for skin and lungs. Asymptomatic patients with mild derangement of LFTs should not be treated. However, patients with symptoms and cholestasis require treatment. Steroids are the first line, though they do not prevent disease progression. Chloroquine, hydroxychloroquine, azathioprine, UDCA, MTX, and infliximab have been used as steroid-sparing agents with chloroquines specifically for cutaneous involvement/hypercalcemia, and MTX and azathioprine for chronic cholestatic disease.