3172 A Rare Complication of Hepatic Cirrhosis: Nephrotic Syndrome in IgA Nephropathy

Abstract

INTRODUCTION: There have been many documented cases of cirrhosis and associated Parenchymal renal disease (Ig-A nephropathy, glomerulonephritis). We discuss here rare case of hepatorenal syndrome type 2, in-patient with no clinically and imaging finding indicative of portal hypertension. Also, further interesting finding is to have nephrotic range proteinuria in nephritic syndrome. CASE DESCRIPTION/METHODS: 56-Year-old Indian male with no past medical or surgical history presented with complaints of generalized weakness and occasional abdominal discomfort. He denied significant family history and alcohol, tobacco or illicit drug use. Ultrasonography (USG) abdomen showed cirrhotic changes. All work up including autoimmune panel negative, hence diagnosed with idiopathic liver cirrhosis. Patient managed with conservative approach, as there were no changes indicative of portal hypertension including Esophagogastroduodenoscopy. After about a year, on routine follow up he found to have his serum creatinine (Cr) progressed from 0.8 to 3.8 mg/dl. Repeat USG abdomen showed diffuse liver parenchymal disease with mild ascites and normal size of kidney with cortical thickness. Urine Microscopy revealed proteinuria and hematuria with 24 hour measured urinary protein 5 gm. Renal biopsy was performed and showed diffuse proliferative glomerulonephritis-Ig A Nephropathy (nephritic syndrome with nephrotic range proteinuria). Autoimmune profile was negative. Hospital course was complicated by septic shock and hemorrhagic cystitis. Patient was treated with inotropes, Pulse dose of intravenous methylprednisolone, albumin and other supportive approach. Decision was made not to continue cyclophosphamide due to hemorrhagic cystitis. Patient Cr later stabilized after peak of 4.3 mg/dl. Presently, is on diuretics and prednisone with Cr is stable around 1.3 mg/dl. DISCUSSION: Hepatorenal syndrome is divesting complication of liver cirrhosis, which can occur due to various mechanism one of which is described as in our case. Literature has focused mainly on mechanism of Ig-A nephropathy in portal hypertension. Our aim for this case report is to focus on monitoring renal function in cirrhotic patient even without no evidence of portal hypertension as there is no firm correlation between degree of portal hypertension and severity of IG A nephropathy. Thus, further studies can effect future era of hepatic transplant by more effectively understanding complex mechanism and managing complications.