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.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
200
Cyclophosphamide
Hôpital LARIBOISIERE
Paris, France
Birmingham Vasculitis Activity Score (BVAS)
Birmingham Vasculitis Activity Score (BVAS)
Time frame: during de study
Chronic lesions (Vasculitis Damage Index)
Chronic lesions (Vasculitis Damage Index)
Time frame: during the study
Renal function at 12 months
Renal function at 12 months
Time frame: during the study
Kidney survival at 12 months
Kidney survival at 12 months
Time frame: during the study
Patient survival at 12 months
Patient survival at 12 months
Time frame: during the study
Blood pressure
Blood pressure
Time frame: during th study
Infections
Infections
Time frame: during the study
Adverse events related to steroid or cyclophosphamide
Adverse events related to steroid or cyclophosphamide
Time frame: during the study
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