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MODERN RHEUMATOLOGY Vol.16 No.2

Vol.16 No.2 に戻る


CASE REPORT

A case of systemic lupus erythematosus complicated by pure red cell aplasia and idiopathic portal hypertension after thymectomy

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Haruyo Iwadate1 , Hiroko Kobayashi1, Kiori Shio1, Etsuko Noguchi1, Kenya Watanabe1, Tomomi Sasajima1, Hideharu Sekine2, Hiroshi Watanabe1, Hiromasa Ohira1, Katsutoshi Obara1 and Yukio Sato1

(1) Department of Internal Medicine II, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima 960-1295, Japan
(2) Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, USA

Received: 17 August 2005 Accepted: 21 February 2006

Abstract We describe a 49-year-old woman who presented in 2002 with pure red cell aplasia (PRCA), systemic lupus erythematosus (SLE), and idiopathic portal hypertension (IPH) that developed following a thymectomy. She underwent a thymectomy at 40 years of age to treat myasthenia gravis. PRCA developed 3 years after the thymectomy and she was successfully treated with cyclosporin. Systemic lupus erythematosus and IPH were diagnosed 6 years later. We conclude that immunological dysfunction resulting from the thymectomy contributed significantly to the subsequent development of PRCA, SLE, and IPH in this patient. This is the first report to describe this extremely rare occurrence.

Key words Idiopathic portal hypertension (IPH) - Pure red cell aplasia (PRCA) - Systemic lupus erythematosus (SLE) - Thymectomy


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