Resolution of Portal Hypertension Following Steroid Therapy for Hepatic Sarcoidosis

Abstract

Purpose: Granulomatous diseases including sarcoidosis are an unusual cause of pre-sinusoidal portal hypertension. We present a case of a patient with both Hepatitis C and sarcoidosis who had significant improvement in portal hypertension following steroid therapy. Methods: A 51 year old Caucasian woman was diagnosed in 2001 with chronic Hepatitis C during a workup for elevated liver enzymes. She was offered treatment with interferon and Ribavirin but she refused. In 2002, she developed peripheral edema, severe anemia, grade 2 esophageal varices and significant splenomegaly. A liver biopsy showed grade 2 hepatitis with stage 3 fibrosis. In 2004, she developed jaundice for the first time and had worsened splenomegaly with secondary pancytopenia. She had gastric varices and grade 3 esophageal varices which required banding. She had never developed encephalopathy. She was offered a TIPS procedure and splenectomy but she came to our institution in 2005 for a second opinion. Laboratory studies showed pancytopenia, mildly elevated liver enzymes, normal albumin and INR. Contrasted CT scan showed extensive abnormal lymphadenopathy in the abdomen, massive splenomegaly, extensive portosystemic varices, mild ascites, normal appearing liver and a spontaneous spleno-renal shunt. Her previous biopsy was reviewed at our institution and showed multiple large granulomas consistent with sarcoidosis along with stage 3 fibrosis. Transjugular liver biopsy was repeated and confirmed the findings. Hepatic venogram showed a hepatic venous pressure gradient (HVPG) of 19 mmHg. Serum angiotensin converting enzyme level was normal. Bone marrow biopsy showed hypercellularity with no evidence of lymphoma. A diagnosis of portal hypertension secondary to sarcoidosis of the liver was made because the fibrosis in the liver was not considered advanced enough to explain the severity of portal hypertension. Results: Prednisone 60 mg a day was started to treat the sarcoidosis and was slowly tapered. Six months later, a repeat hepatic hemodynamic study showed HVPG of 7 mmHg, while still on Prednisone 20 mg a day. CT showed resolution of ascites and repeat endoscopy showed no esophageal or gastric varices. In one year, HVPG decreased further to 5 mmHg. Currently, she is on Prednisone 2.5 mg a day without any further worsening of her portal hypertension. Conclusion: This is a patient with non cirrhotic stage Hepatitis C who had portal hypertension out of proportion to her chronic viral liver disease. She was found to have extensive sarcoidosis in the liver which ultimately was treated with steroids and the portal hypertension resolved. This report supports the use of steroid therapy in patients with sarcoidosis who have portal hypertension.