Henoch-Schönlein Purpura Nephritis: Pathophysiology,... : Clinical Journal of the American Society of Nephrology (original) (raw)
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Henoch-Schönlein Purpura Nephritis
Pathophysiology, Treatment, and Future Strategy
Department of Pediatric Nephrology, Emma Children's Hospital-Academic Medical Center, Amsterdam, The Netherlands and Department of Pediatric Nephrology, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium
Correspondence: Dr. J. C. Davin, Pediatric Nephrology Department, Emma Children's Hospital-Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam Z-O, The Netherlands. Tel: 31205669111; Fax: 31206917735; E-mail: [email protected]
Clinical Journal of the American Society of Nephrology 6(3):p 679-689, March 2011. | DOI: 10.2215/CJN.06710810
Abstract
Summary
Henoch-Schönlein purpura nephritis is a rare kidney disease leading to chronic kidney disease in a non-negligible percentage of patients. Although retrospective studies suggest beneficial effects of some therapies, prospective randomized clinical trials proving treatment efficacy are still lacking. The dilemma of spontaneous recovery even in patients with severe clinical and histologic presentation and of late evolution to chronic kidney disease in patients with mild initial symptoms renders it difficult for clinicians to expose patients to treatment protocols that are not evidence-based. A better understanding of the pathophysiology of progression to chronic kidney disease in Henoch-Schönlein purpura patients could be achieved by designing prospective international multicenter studies looking at determinants of clinical and histopathological evolution as well as possible circulating and urinary markers of progression. Such studies should be supported by a database available on the web and a new histologic classification of kidney lesions. This paper reports clinical, pathologic, and experimental data to be used for this strategy and to assist clinicians and clinical trial designers to reach therapeutic decisions.
Copyright © 2011 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
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