Efficacy and Safety of Imatinib in Scleroderma

This study will test the efficacy and tolerance of patients with a high score of induration (modified Rodnan score > 20/54) Comparison : 34 patients with severe forms of cutaneous involvement will be evaluated in a double blind RCT comparing imatinib 400mg/j and placebo in a 6 month period. Efficacy will be assessed using a cutaneous induration scale and skin biopsy, and quality of life questionnaires.

Inclusion criteria

• More than 18 years old
• Documented diagnostic of scleroderma (systemic or cutaneous)
• Severe cutaneous sclerodermia or systemic sclerodermia with m-Rodnan score > 20/51
• Ejection fraction of more than 45 per cent at cardiac ultrasound pre-inclusion study
• Woman with efficient contraceptive method during trail treatment and during 3 month after the end of trial treatment
• All female patients with reproductive potential must have a negative pregnancy test (serum or urine) within the 7 days prior to enrolment
• Affiliated or profit patient of a social security system
• Signed informed consent

Exclusion criteria

• new systemic treatment, potentially interfering with disease progression, beginning 3 months prior the start trial treatment
• Patient with isolated cutaneous scleroderma treated with a drug potentially interfering with the course of the disease 4 weeks before starting the trial (Systemic corticosteroids, methotrexate, cyclophosphamide, bosentan)
• Scleroderma " en coup de sabre "
• Severe organ failure or anomaly of blood chemistry/hematology (bilirubin, SGOT, SGPT, creatinine > 1,5 ´ upper normal limit, polymorphonuclear granulocytes less than 1*10*9/l or platelets less than 50*10*9/l),
• Ongoing cancer
• Ejection fraction ≤ 45 per cent at cardiac ultrasound pre inclusion study
• myocardial infarction of less than 6 mois at pre inclusion visit
• Non controlled chronic illness (diabetes, chronic kidney failure, chronic hepatitis, HIV infection),
• Major surgery less than two weeks before inclusion
• Pregnancy or lactation
• Absence of validated contraception in childbearing women.
• Contraindication to imatinib mesylate treatment as specified in product specifications
• Non observance anticipated and absence of informed consent