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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| 1 | Experimental | Cyclophosphamide |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Cyclophosphamide | Drug | Cyclophosphamide |
|
| Measure | Description | Time Frame |
|---|---|---|
| Birmingham Vasculitis Activity Score (BVAS) | Birmingham Vasculitis Activity Score (BVAS) | during de study |
| Measure | Description | Time Frame |
|---|---|---|
| Chronic lesions (Vasculitis Damage Index) | Chronic lesions (Vasculitis Damage Index) | during the study |
| Renal function at 12 months | Renal function at 12 months |
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Inclusion Criteria:
Exclusion criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Eric THERVET, MD | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital LARIBOISIERE | Paris | 75015 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 11961015 | Result | 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. | |
| 36853224 | Derived | Hahn D, Hodson EM, Craig JC. Interventions for preventing and treating kidney disease in IgA vasculitis. Cochrane Database Syst Rev. 2023 Feb 28;2(2):CD005128. doi: 10.1002/14651858.CD005128.pub4. |
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| ID | Term |
|---|---|
| D011695 | IgA Vasculitis |
| D014657 | Vasculitis |
| D051437 | Renal Insufficiency |
| ID | Term |
|---|---|
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
| D011693 | Purpura |
| D001778 | Blood Coagulation Disorders |
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| ID | Term |
|---|---|
| D003520 | Cyclophosphamide |
| ID | Term |
|---|---|
| D010752 | Phosphoramide Mustards |
| D009588 | Nitrogen Mustard Compounds |
| D009150 | Mustard Compounds |
| D006846 | Hydrocarbons, Halogenated |
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| during the study |
| Kidney survival at 12 months | Kidney survival at 12 months | during the study |
| Patient survival at 12 months | Patient survival at 12 months | during the study |
| Blood pressure | Blood pressure | during th study |
| Infections | Infections | during the study |
| Adverse events related to steroid or cyclophosphamide | Adverse events related to steroid or cyclophosphamide | during the study |
| 20505654 | Derived | 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. |
| D006402 |
| Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D020141 | Hemostatic Disorders |
| D006474 | Hemorrhagic Disorders |
| D017445 | Skin Diseases, Vascular |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D007105 | Immune Complex Diseases |
| D006967 | Hypersensitivity |
| D007154 | Immune System Diseases |
| D006470 | Hemorrhage |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D012877 | Skin Manifestations |
| D012816 | Signs and Symptoms |
| D007674 | Kidney Diseases |
| D014570 | Urologic Diseases |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
| D052801 | Male Urogenital Diseases |
| D006838 | Hydrocarbons |
| D009930 | Organic Chemicals |
| D063088 | Phosphoramides |
| D009943 | Organophosphorus Compounds |