Discovery Of Granulomatous Hepatitis After Withdrawal Of Steroids

INTRODUCTION: We describe a case of granulomatous hepatitis presenting with decompensated cirrhosis and portal hypertension after withdrawal of steroids. CASE DESCRIPTION/METHODS: 74 year old Caucasian male with type 2 diabetes and anemia reported chronic fatigue. He did not report any associated symptoms such as fevers, chills, chronic cough, joint pains, redness or swelling in his hands, lumps in his neck, skin color changes in the cold or any rashes. On exam, he was anicteric with hepatosplenomegaly. His labs showed pancytopenia (Hgb 10.4 g/dL, platelets 136 K/cmm and WBC 4.3/uL), iron deficiency (low ferritin of 27 ng/mL and low iron of 34 ug/dL) and elevated ALP (470 U/L) with an elevated GGT (515 U/L). He had an elevated Angiotensin Converting Enzyme level (88 nmol/mL/min), elevated ESR (53 mm/hr) and negative ANA; tuberculin skin test was negative. CT imaging showed sub-5 mm resolved thoracic calcified granulomas and hepatosplenomegaly with splenic lesions. A bone marrow biopsy for evaluation of pancytopenia showed noncaseating granulomas without any evidence of lymphoma or any other hematologic malignancy (Figure 1); acid fast bacilli stain was negative. Given clinical suspicion of extra-pulmonary sarcoidosis of the bone marrow and spleen, rheumatology started patient on prednisone. Prednisone led to mild improvement in fatigue and decreased ALP to 154 U/L. As the patient experienced complications of hyperglycemia and the diagnosis of extra-pulmonary sarcoidosis was not clearly established in a relatively asymptomatic patient, rheumatology discontinued prednisone after 9 months. Few months later, he reported abdominal distension and pruritus. Exam was noted for massive ascites. His labs were suggestive of portal hypertension and hepatic decompensation (platelets of 82 K/cmm, albumin of 3.3 g/dL and ALP of 387 U/L). Liver core biopsy showed cirrhosis in the setting of granulomatous hepatitis (Figure 2). Patient was restarted on prednisone; ursodeoxycholic acid was also added. His ascites and labs improved (albumin increased to 3.9 g/dL and ALP decreased to 93 U/L). Attempts to transition patient to immunomodulators were unsuccessful as patient was deemed to be a heterozygote metabolizer of thiopurine methyltransferase and reported lethargy during a trial of methotrexate. DISCUSSION: For any patient with labs suggestive of hepatic involvement of sarcoidosis or granulomatous disease, strong consideration should be given to performing liver biopsy prior to starting treatment.Figure 1.: Bone marrow biopsy showed non-caseating epithelioid granulomas with multi-nucleated giant cells.Figure 2.: Liver biopsy showed several confluent non-caseating granulomas composed of epithelioid cells derived from macrophages.