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To assess the efficacy of systemic corticosteroids alone as first-line treatment of Churg-Strauss syndrome without poor-prognosis factors as defined by the five-factor score (FFS=0), and to compare the efficacy and safety of azathioprine vs pulse cyclophosphamide as adjunctive immunosuppressive therapy to treat failure or relapse
All patients initially treated with systemic corticosteroids alone: optional IV methylprednisolone pulse (15 mg/kg) at treatment start followed by oral prednisone (1 mg/kg/day) according to a tapering schedule. Treating physicians allowed to treat minor relapses with corticosteroids without referring the patient for randomization, as long as the prednisone dose did not exceed 0.5 mg/kg for 1 month.
Patients in whom prednisone doses could not be tapered below 20 mg, those who failed to enter remission and those who relapsed were randomized to receive either 6 months of oral azathioprine (2mg/kg/day) or 6 cyclophosphamide pulses (0.6g/m2 D1, D15, D30 then every month)
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| azathioprine | Drug | |||
| cyclophosphamide | Drug |
| Measure | Description | Time Frame |
|---|---|---|
| Number of events (failures, relapses and/or deaths) occurring in each group, defining the disease-free survival rate, measured at study end (mean follow-up of 5 years) |
| Measure | Description | Time Frame |
|---|---|---|
| Overall survival, relapse rate and adverse events, measured at study end (mean follow-up of 5 years) |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Jean-François Cordier, MD | Hospices Civils de Lyon | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Camillo Ribi | Geneva | Switzerland |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 25153486 | Derived | Samson M, Puechal X, Devilliers H, Ribi C, Cohen P, Bienvenu B, Terrier B, Pagnoux C, Mouthon L, Guillevin L; French Vasculitis Study Group (FVSG). Mononeuritis multiplex predicts the need for immunosuppressive or immunomodulatory drugs for EGPA, PAN and MPA patients without poor-prognosis factors. Autoimmun Rev. 2014 Sep;13(9):945-53. doi: 10.1016/j.autrev.2014.08.002. Epub 2014 Aug 19. | |
| 23590801 |
| Label | URL |
|---|---|
| Related Info | View source |
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| ID | Term |
|---|---|
| D015267 | Churg-Strauss Syndrome |
| D014657 | Vasculitis |
| ID | Term |
|---|---|
| D056648 | Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis |
| D056647 | Systemic Vasculitis |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |
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| ID | Term |
|---|---|
| D001379 | Azathioprine |
| D003520 | Cyclophosphamide |
| ID | Term |
|---|---|
| D013872 | Thionucleosides |
| D013457 | Sulfur Compounds |
| D009930 | Organic Chemicals |
| D015122 | Mercaptopurine |
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| Derived |
| Samson M, Puechal X, Devilliers H, Ribi C, Cohen P, Stern M, Pagnoux C, Mouthon L, Guillevin L; French Vasculitis Study Group. Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun. 2013 Jun;43:60-9. doi: 10.1016/j.jaut.2013.03.003. Epub 2013 Apr 13. |
| 18240234 | Derived | Ribi C, Cohen P, Pagnoux C, Mahr A, Arene JP, Lauque D, Puechal X, Letellier P, Delaval P, Cordier JF, Guillevin L; French Vasculitis Study Group. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008 Feb;58(2):586-94. doi: 10.1002/art.23198. |
| D006099 | Granuloma |
| D008232 | Lymphoproliferative Disorders |
| D008206 | Lymphatic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D017445 | Skin Diseases, Vascular |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D001327 | Autoimmune Diseases |
| D007154 | Immune System Diseases |
| D011687 |
| Purines |
| D006574 | Heterocyclic Compounds, 2-Ring |
| D000072471 | Heterocyclic Compounds, Fused-Ring |
| D006571 | Heterocyclic Compounds |
| D009705 | Nucleosides |
| D009706 | Nucleic Acids, Nucleotides, and Nucleosides |
| D010752 | Phosphoramide Mustards |
| D009588 | Nitrogen Mustard Compounds |
| D009150 | Mustard Compounds |
| D006846 | Hydrocarbons, Halogenated |
| D006838 | Hydrocarbons |
| D063088 | Phosphoramides |
| D009943 | Organophosphorus Compounds |