Journal of Gastroenterology and Hepatology (1999) 14, 431–435 She was known to have had PBC since 1988, estab- lished by biochemical cholestasis (alkaline phosphatase (ALP) three to four times the upper limit of normal and gamma glutamyltransferase (GGT) 15–20-fold the upper limit of normal), high total serum bile acid levels of 49 mmol/L (normal < 9), high titre of anti-M 2 (1:160) and, subsequently, by a compatible core liver biopsy (performed on 16 July 1993) showing nodularity, portal–portal bridging fibrosis, severe piecemeal necro- sis, marked bile duct damage with presence of plasma cells and lymphocytes in the portal tracts; and on mod- ified orcein staining, very marked deposition of copper- related proteins at the lobular margins (Figs 1,2). Levels of serum copper, caeruloplasmin, a 1 -anti- trypsin and iron studies were within normal ranges. Serology for hepatitis A virus, hepatitis B virus and hepatitis C virus were negative. In June 1993, her ANA titre was positive at 1:80 (speckled) but the anti-dsDNA was negative. The liver function tests remained unchanged until 6 months prior to her presentation, when her serum bilirubin had risen to 50 mmol/L and albumin dropped to 32 g/L. Her past history included paranoid schizophrenia needing one admission to a psychiatric hospital at the age of 27 years and which had been treated intermit- tently by Stelazine (trifluoperazine) since July 1987 (usually at a low dose between 1 and 5 mg/day because the patient refused to stop it for fear of recurrence of INTRODUCTION Both primary biliary cirrhosis (PBC) and systemic lupus erythematosus (SLE) are rare diseases. Although the former is accompanied by an autoimmune illness or its markers in approximately 80% of cases, 1 associations between PBC and SLE are extremely uncommon. 2 We report a patient with confirmed PBC who, after 10 years of follow up, developed classical signs and symptoms of SLE in the form of polyserositis, notably pericardial tamponade, a typical photosensitive skin rash, high titres of anti-nuclear antibodies (ANA) and anti-double stranded DNA (anti-dsDNA) and antibodies to Ro antigen and Smith antigen (Sm). CASE REPORT A 57-year-old lady of Greek descent presented to the hospital on 10 March 1998 with non-specific abdomi- nal and left-sided pleuritic chest pain for 3 days. She had a temperature varying between 37 and 38.5°C, pleuritic type chest pain but no pleural rub and no clin- ical evidence of deep venous thrombosis in the lower limbs. Her echocardiogram was within normal limits. The chest X-ray and total white cell count were normal but there was persistent thrombocytopenia from her previously known hypersplenism secondary to her PBC. HEPATOBILIARY DISEASES CASE REPORT: A rare association of primary biliary cirrhosis and systemic lupus erythematosus and review of the literature SHAMSUL ISLAM,* JOHN W RIORDAN † AND JENNIFER A MCDONALD* *Departments of Gastroenterology and † Rheumatology,Wollongong Hospital, Illawarra, NSW, Australia Abstract We report a rare occurrence of systemic lupus erythematosus in a patient known to have had well-documented primary biliary cirrhosis for 10 years. The presentation was dramatic with peri- cardial tamponade, but responded well to high dose corticosteroid. There are only five such definite associations reported in the literature. In the present case, other possible causes were considered, such as drug-induced cholestasis, drug-induced lupus, autoimmune chronic active hepatitis and the overlap syndrome. © 1999 Blackwell Science Asia Pty Ltd Key words: corticosteroid response, pericardial tamponade, primary biliary cirrhosis, systemic lupus erythematosus. Correspondence: Jennifer A McDonald, Department of Gastroenterology,Wollongong Hospital,Wollongong, NSW 2500, Aus- tralia. Email: <islam@ozemail.com.au> Accepted for publication 30 November 1998.