Treatment of patients with Nephrogenic Systemic Fibrosis(NSF) with Imatinib Mesylate(Glivec). An open label clinical trial among patients with moderate to severe NSF.

• Conditions: ephrogenic Systemic Fibrosis (NSF) is a fibrosing disease strongly associated with Gadolinium Based Contrast Agents(GBCA). Hard dermal plaques appear on legs, arms and abdomen. The lesions involve the deep part of the subcutis. The skin hardens and loses flexibility. The muscles are often affected too. Involvement of the joint capsulaes leads to contractures. Some patients ends up in a wheelchair. The inner vital organs may be affected and NSF can accelerate the patients death.; MedDRA version: 12.0Level: LLTClassification code 10067467Term: Nephrogenic systemic fibrosis

• Registration Number: EUCTR2009-013295-48-DK
• Lead Sponsor: Århus University Hospital, Department of Dermatology

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Study Start: 2009-07-21
• Last Update Posted: 19 March 2012

Recruitment & Eligibility

• Status: ot Recruiting
• Sex: All
• Target Recruitment: Not specified

Inclusion Criteria

1.Age > 18 years
2.Diagnosed with NSF
3.mRodnan skinscore => 20 or
4.Rapid progression of the disease defined as a 50 % increase in mRodnan skin score in less than 7 weeks or
5.Progression of the fibrosis in the inner organs and
6.No absolute contraindications to the treatment.

Are the trial subjects under 18? no
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) yes
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

Exclusion Criteria :

1.Known sensitivity to Imatinib Mesylate or to any of its components
2. Pregnant or lactating woman
3.ALAT > 3 x upper limit of normal
4. Severe congestive heart failure(NYHA Class III or IV)

Study & Design

• Study Type: Interventional clinical trial of medicinal product
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Main Objective: To offer the NSF patients with moderate to severe disease treatment with imatinib mesylate (Glivec) as no treatment so far has shown to be acceptable effective.<br>Primary measurement will be evaluation of the skin with the mRodnan skinscore<br>;Secondary Objective: To follow these patients very closely with bloodsamples, biopsies, mRodnan skinscore and DLQI in order to control if the treatment is well tolerated and is effective to the patient. <br>Skinbiopsies to see if there is any histopathological changes.<br>DLQI to follow any changes in quality of life;Primary end point(s): To decelerate the progression of the disease and thereby soften the skin and improve the mobility of the joint contractures. <br>One case report by Jonathan Kay has shown beneficial effect of Imatinib mesylate in 2 patients at the Massachusetts General Hospital.<br>We hope that our studie can bring more knowledge about the treatment of this group of patients, whom today have very few treatment options.