A Case of Isolated Hepatosplenic Sarcoidosis

Abstract

Purpose: Sarcoidosis, a granulomatous disorder of unknown etiology, primarily affects the lungs and lymph nodes. Extra-pulmonary disease without any pulmonary involvement is rare. We describe a case of isolated hepatosplenic sarcoidosis, confirmed by biopsy. Results: A 49 year-old African American female with no significant past medical history was found to have hepatomegaly on exam at an urgent care center. She initially presented with headache, 20-lb weight loss in six months, and subjective chills. On physical exam, the liver was palpable to 6 cm below the costal margin. There was no jaundice or stigmata of chronic liver disease. The rest of the physical exam was unremarkable. She was admitted for workup, as we were concerned for malignancy. Initial laboratory studies showed AST of 76 U/L, alkaline phosphatase of 533 U/L and GGT of 513 U/L. ACE level was elevated at 131 IU/L. Flow cytometry did not reveal any evidence of lymphoma. Abdominal ultrasound revealed multiple echogenic masses in the liver with indistinct borders and periportal distribution. Abdominal and chest CT scan showed diffuse areas of hypoattenuation in the liver and spleen, occupying 25% and 75% of the hepatic and splenic parenchyma, respectively. Adenopathy was present in the retroperitoneum but not in the thorax. Liver biopsy revealed extensive areas of fibrosis with multiple epithelioid cell granulomas, consistent with sarcoidosis. The patient was referred to our Sarcoid specialty clinic for follow-up. Conclusion: We present a rare case of hepatosplenic sarcoidosis without any evidence of pulmonary or mediastinal involvement. Diagnosis of hepatic sarcoidosis is important as a minority of cases may progress to end-stage liver disease.Figure