A case of systemic lupus erythematosus complicated by pure red cell aplasia and idiopathic portal hypertension after thymectomy |
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Authors: | Haruyo Iwadate Hiroko Kobayashi Kiori Shio Etsuko Noguchi Kenya Watanabe Tomomi Sasajima Hideharu Sekine Hiroshi Watanabe Hiromasa Ohira Katsutoshi Obara Yukio Sato |
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Institution: | (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 |
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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. |
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Keywords: | Idiopathic portal hypertension (IPH) Pure red cell aplasia (PRCA) Systemic lupus erythematosus (SLE) Thymectomy |
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