Treatment with high dose Methotrexate in patients with generalized eosinophilic fasciitis
- Conditions
- Shulman's syndrome or diffuse fasciitis10010761
- Registration Number
- NL-OMON30014
- Lead Sponsor
- niversitair Medisch Centrum Sint Radboud
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Pending
- Sex
- Not specified
- Target Recruitment
- 6
eosinophilic fasciitis as defined by clinical judgement of an expert, histologically confirmed with a skin-fascia-muscle biopsy, and in addition:
Either the presence of a modified skin score according to Zachariae of 8 or higher (0-18), regarded as a generalised eosinophilic fasciitis or the presence of severe contractures due to eosinophilic fasciitis lesions
Age < 18 yrs
Contraindications to MTX: AST/ALT level > 2 times upper limit of normal, WBC count < 3.5 x 109/l or platelet count < 150 x 109/l, serum creatinine > 130 micromol/l or clearance < 50 ml/min, confirmed by two repeated tests within one month
Chronic liver disease, insulin dependant diabetes mellitus, alcohol abuse
Restrictive pulmonary disease (total lung capacity or vital capacity < 40% of predicted) or interstitial lung disease (KCO < 60% of predicted)
Pregnancy or child bearing potential without adequate contraception
The presence of any serious co-morbidity or malignancy
Use of other anti-folate drugs than MTX
Study & Design
- Study Type
- Interventional
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method <p>Score of involved skin with a modified skin score according to Zachariae and<br /><br>assessed with a durometer </p><br>
- Secondary Outcome Measures
Name Time Method <p>The amount of skin induration observed as redness of involved areas.<br /><br>Range of motions of involved joints<br /><br>Peripheral blood eosinophilia, immunoglobulines and ESR<br /><br>Visual analogue scale of disease activity by patient and doctor<br /><br>A health assessment questionnaire (SF-36)<br /><br>Chest expansion<br /><br>Pulmonary function (total lung capacity, vital capacity, FEV1 and KCO)</p><br>