Shulman's syndrome or diffuse fasciitis
Conditions
Interventions
MTX is administered in a dose of 4 mg/kg (in 250 ml 0.9% NaCl in 30 minutes)
intravenously on the day-care unit of the Department of Rheumathology every
four weeks. Twenty-four hours after the admin
25 mg orally in 5 doses every
6 hours. This treatment schedule is based upon protocols used in the Department
of Hematology for the treatment of acute lymphoblastic leukemia.
Sponsors
Universitair Medisch Centrum Sint Radboud
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: 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
Exclusion criteria
Exclusion criteria: Age 2 times upper limit of normal, WBC count 130 micromol/l or clearance
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Score of involved skin with a modified skin score according to Zachariae and assessed with a durometer | — |
Secondary
| Measure | Time frame |
|---|---|
| The amount of skin induration observed as redness of involved areas. Range of motions of involved joints Peripheral blood eosinophilia, immunoglobulines and ESR Visual analogue scale of disease activity by patient and doctor A health assessment questionnaire (SF-36) Chest expansion Pulmonary function (total lung capacity, vital capacity, FEV1 and KCO) | — |
Countries
Netherlands
Outcome results
None listed