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CASE REPORT
Normotensive scleroderma renal crisis with diffuse alveolar damage after corticosteroid therapy

Taio Naniwa1 , Shogo Banno1, Nobuyuki Takahashi1, Shinji Maeda1, Yoshihito Hayami1 and Ryuzo Ueda1

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(1) Division of Rheumatology, Department of Internal Medicine and Molecular Science, Nagoya City University Graduate School of Medical Sciences, Kawasumi, Mizuho-ku, Nagoya 467-8601, Japan

Received: 15 September 2004 Accepted: 24 December 2004

Abstract A 68-year-old woman with systemic sclerosis developed acute respiratory failure due to diffuse alveolar hemorrhage and normotensive scleroderma renal crisis (SRC) shortly after the initiation of corticosteroid therapy. Treatment with angiotensin-converting enzyme inhibitor and plasmapheresis had failed in this patient. Autopsy showed diffuse alveolar damage and thrombotic micro-angiopathy. The sequence of events in this patient clarifies the pathologic process of normotensive SRC, and suggests a causative role of corticosteroid therapy and normotensive SRC.

Key words Corticosteroid - Diffuse alveolar damage (DAH) - Scleroderma renal crisis (SRC) - Systemic sclerosis (SSc) - Thrombotic microangiopathy (TMA)


 

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