โ Scribed by R P Perrillo; A L Mason; S Jacob; M A Gerber
No coin nor oath required. For personal study only.
mal in size and echogenicity. Ascites and splenomegaly were absent. A hepatitis panel and immunoserological tests re-The patient is a 42-year-old female who presented to a local vealed the following: hepatitis A virus antibody /:antihospital in September of 1994 with a history of scleral icterus. nuclear antibody (ANA), /1:2,560; hepatitis B surface anti-She first noticed this in July but did not seek medical advice gen 0:AMA, /1:320; hepatitis B surface antibody 0:ASMA, at that time. The jaundice progressed, and she developed gener-/1:20; hepatitis B core antibody /:Anti-liver/kidney microalized weakness and malaise that increased over several weeks. somal antibody, /1:40; hepatitis B e antigen 0:immunoglob-The patient eventually became homebound because of her faulin G, 2,050 mg/dL (normal range, 725-1,475 mg/dL); hepatitigue. Her other complaints were decreased appetite and protis B e antibody 0:immunoglobulin A (IgA), 511 mg/dL gressive swelling of the lower extremities. When she was seen (normal range, 86-360 mg/dL); and hepatitis C virus antibody by a local physician in mid September, her laboratory data 0:IgM, 356 mg/dL (normal range, 79-463 mg/dL). revealed an alanine transaminase of 360 U/L, aspartate trans-
The presence of circulating antibody to pyruvate dehydroaminase of 1215 U/L (normal ranges, 5 to 40 U/L), total bilirubin genase subunit 2 was detected by both an ELISA and Westof 17.5 mg/dL (normal range, .2 to 1.5 mg/dL), and an alkaline ern blot reactivities (kindly performed by Dr. Michael Manns, phosphatase of 285 U/L (normal range, 38 to 126 U/L). Her Hannover, Germany). physician recommended prednisone therapy at a dose of 40 mg
The patient underwent liver biopsy in October, 1994, which daily. She was referred to the Ochsner Clinic (New Orleans, will be described below. LA) on October 15, 1994, approximately 3 weeks after initiating She was initially maintained on 40 mg of prednisone and immunosuppressive therapy. 600 mg of ursodeoxycholic acid daily was added. Her liver Her past medical history was significant for vaginal bleedtests improved and 50 mg of azathioprine daily was initiated ing in November of 1993, which required transfusions and a 2 months later enabling reduction of the prednisone dose to hysterectomy. She had no other risk factors for liver disease. 30 mg per day. At that time, serum cholesterol had risen to She also had a history of hypothyroidism for approximately 332 mg/dL, and she was started on insulin therapy because 1 year and was on thyroid replacement therapy. Her family of new onset hyperglycemia. Two months later, her corticostehistory was significant for diabetes and hypothyroidism. roid dose was reduced to 25 mg and azathioprine was in-When seen at the Ochsner Clinic, she was noted to be jauncreased to 75 mg. During this time, her liver tests were steaddiced and there was palmar erythema with no other visible ily improving (Fig. and). Five months after starting evidence of chronic liver disease. The liver edge was palpable treatment, her azathioprine dose was further increased to just below the costal margin with a span of 10 cm. The spleen 100 mg and 2 months later the prednisone dose was reduced was not palpable and there was no evidence of ascites. The to 10 mg per day. Over the next 5 months her prednisone patient had 1/ pedal edema in her lower extremities.
dose was further reduced to 2.5 mg and azathioprine dose Laboratory data were as follows: alanine transaminase, was increased to 150 mg. One year after initial diagnosis the 244 U/L (normal range, 5-40 U/L); aspartate transaminase, azathioprine was increased to 175 mg (approximately 2 mg/ 834 U/L (normal range, 5-40 U/L); total bilirubin, 18.2 mg/ kg) and she was maintained on 2.5 mg of prednisone, but the dL (normal range, 1-1.0 mg/dL); alkaline phosphatase, 347 azathioprine dose had to be reduced because of neutropenia. U/L (normal range, 45-130 U/L); gamma glutamyl transpepti-She had a repeat liver biopsy performed in October of 1995, dase, 316 U/L (normal range, 8-55 U/L); albumin, 2.6 gm/dL which is described below. One month later, her liver function (normal range, 3.5-5.5 gm/dL); alpha 1 Antitrypsin, 305 mg/ tests continued to show improvement with an aspartate dL (normal range, 190-350 mg/dL); hemoglobin, 9.9 gm/dL transaminase of 89 U/L, an ALT of 54 U/L, and alkaline (normal range, 14-18 gm/dL); white blood count, 5.9 1 10 3 / phosphatase of 186 U/L. The patient continues to feel well mm 3 (normal range, 4,800-10,800/mm 3 ); platelets, 228,000/ and has resumed full duties. mm 3 (normal range, 150,000-350,000/mm 3 ); prothrombin time, 17.9 sec (normal range, 10.5-12.5 sec); serum iron, 120 DISCUSSION mcg/dL (normal range, 45-160 mcg/dL); total iron binding Robert Perrillo, M.D. The differential diagnosis of acute capacity, 145 mcg/dL (normal range, 228-428 mcg/dL); and hepatocellular injury with mixed features of hepatitis and ferritin, 2,454 mg/dL (normal range, 40-300 mg/dL).
cholestasis includes consideration of acute viral hepatitis, al-An abdominal ultrasound revealed that the liver was norcoholic liver injury, medication induced liver disease, primary biliary cirrhosis (PBC), and autoimmune hepatitis (AIH).
Most of these can be easily excluded in this case, because the patient was not a drinker, nor had she taken any medications Abbreviations: ANA, antinuclear antibody; PBC, primary biliary cirrhosis; AIH, autoimmune hepatitis; AMA, antimitochondrial antibody; SMA, smooth muscle antibody; PDC-except thyroxine. While there was a history of transfusions, E2, pyruvate dehydrogenase complex; LKM-1, liver kidney microsomal-1. her hepatitis profile was negative for hepatitis B and C vi-From the
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