Efficacy of Steroid Versus Steroid Plus Cyclophosphamide for Severe Henoch-Schoenlein Purpura

CESAR (Randomized Therapeutic Study of Steroid vs. Steroid Plus Cyclosphosphamide for Severe Viscera Henoch-Schoenlein Purpura)

Henoch-Schonlein purpura is a leucocytoclastic systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It is characterized by the association of skin, joint and gastrointestinal manifestations. Even though the evolution is usually simple, some patients, especially adults, may have severe visceral involvement including heart, lung, brain and renal disease. The best treatment is currently unknown. This study will test the safety and efficacy of steroids associated or not with cyclosphosphamide to treat the acute lesions and to prevent the development of chronic lesions.

The protocol test the role of 2 modalities of treatment (steroid vs. steroid and cyclophosphamide) for severe systemic form of Henoch-Schonlein purpura. The severe forms include extracapillary glomerulonephritis, myocarditis, pneumonitis, occult involvement, severe gastro-intestinal bleeding. No other randomized trial has been performed in adult patient for this indication.

Inclusion Criteria: Patients with Henoch-Schoenlein purpura Patient's age > 18 years Exclusion criteria: Patient presenting a purpura RHEUMATOID the diagnosis of which is confirmed by the histology presenting at least a visceral infringement(achievement) making consider the affection as engraves(burns) of 18 or more years old Patient Patient capable of understanding(including) the advantages and the risks of the try Patient having given his assent lit in writing

Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schonlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002 May;13(5):1271-8. doi: 10.1097/01.asn.0000013883.99976.22.

Pillebout E, Alberti C, Guillevin L, Ouslimani A, Thervet E; CESAR study group. Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schonlein Purpura. Kidney Int. 2010 Sep;78(5):495-502. doi: 10.1038/ki.2010.150. Epub 2010 May 26.