Rahul Patel, DO1, Anudeep Jala, DO1, Matthew Everwine, DO1, Smita Patel, MD1, Cindy Hou, DO1, Lucy Joo, DO2 1Jefferson Health, Washington Township, NJ; 2Jefferson Health, Cherry Hill, NJ
Introduction: The etiology of sarcoidosis is a mystery but genetic predisposition, exposure of substances associated with granuloma formation and infectious agents are believed to play a role. We report a case of hepatic sarcoidosis associated with hypogammaglobulinemia and hepatitis B.
Case Description/Methods: A 37-year-old male with a history of childhood IgG/IgA deficiency on monthly intravenous immunoglobulin (IVIG) with recurrent infectious hospitalizations, prior intravenous drug abuse on methadone, tobacco abuse, type 1 diabetes and hepatitis B was referred to gastroenterology for evaluation of recurrent nausea and vomiting. His labwork showed alkaline phosphatase (ALP) 935 IU/L, aspartate transaminase (AST) 64 IU/L, alanine transaminase (ALT) 50 IU/L, gamma glutamyl transferase (GGT) 173 IU/L, unremarkable bilirubin and pancytopenia. He follows hematology and recently had a bone marrow biopsy demonstrating minute noncaseating granulomas with no malignancy. Angiotensin converting enzyme (ACE) was 159. Viral hepatitis panel demonstrated prior resolved Hepatitis B infection. CT abdomen/pelvis with intravenous contrast showed hepatic steatosis, splenomegaly and bilateral lung nodules. Nonalcoholic steatohepatitis (NASH) fibrosure plus study demonstrated F1 portal fibrosis S2-S3. Given the recent bone marrow biopsy findings, elevated ACE and abnormal hepatic panel he underwent liver biopsy which demonstrated intact lobular architecture, moderate chronic lymphocytic inflammation of the portal tract and liver lobular parenchyma and scattered non necrotizing granulomas consistent with hepatic sarcoidosis. He was started on ursodiol 300mg twice a day. His nausea/vomiting was suspected to be from diabetic gastroparesis so he was set up for a gastric emptying study but endoscopic evaluation was deferred due to leukopenia. After multidisciplinary discussion involving gastroenterology, infectious diseases, rheumatology and hematology, he was started on a 20-day prednisone taper.
Discussion: The worldwide prevalence of sarcoidosis is 2-60 per 100,000 people. 80% of afflicted individuals have lung involvement. Our patient was evaluated by pulmonology with pulmonary involvement ruled out. While the exact mechanism of our patient’s sarcoidosis etiology is unknown, it is multifactorial from hepatitis B and hypogammaglobulinemia which is rare. This challenging diagnostic case highlighted the importance of multi-specialty collaboration for optimal patient care.
