Health Condition 1: M330- Juvenile dermatomyositis
Conditions
Interventions
Intervention1: Tofacitinib: Eligible children will be enrolled in the study and will be given oral tofacitinib (intervention) in the recommended doses as per weight band by EMA (European Medicines age
Sponsors
Department of Pediatrics, AIIMS, New Delhi
Eligibility
Inclusion criteria
Inclusion criteria: 1Children age 2 to 18 years with -Juvenile Dermatomyositis (JDM) OR Juvenile Dermatomyositis Systemic Scleroderma overlap (JDM-SSc) AND -calcinosis cutis on physical examination or previous imaging.
Exclusion criteria
Exclusion criteria: 1Patient cyclophosphamide during the preceding 1 month or rituximab in the last 6 months OR -Topical tacrolimus OR - Active Tuberculosis OR - Hemoglobin (Hb) levels less than 9 g per dL OR - Absolute lymphocyte count less than 500 cells per mm3 -Absolute neutrophil count less than 1000 cells per mm3
Design outcomes
Secondary
| Measure | Time frame |
|---|---|
| 1)Proportion of children demonstrating improvement in clinical scores ( CDASI and CMAS) 2)Number of subjects with adverse events. 3)Change in doses(mean) of steroids 4)Correlation of baseline laboratory parameters ( myositis specific antibody and interferon levels) with outcome.Timepoint: 1) At baseline and 24 ±2 week follow-up 2)Anytime from initiation of therapy to 24 ±2 weeks after initiation of therapy. 3)Anytime from initiation of therapy to 24 ±2 weeks after initiation of therapy. 4)At baseline and 24 ±2 week follow-up | — |
Primary
| Measure | Time frame |
|---|---|
| Effect of Tofacitinib on calcinosis cutis in children with JDM as assesed by reduction in Agatston score using ultra low-dose whole body CT scan.Timepoint: At baseline and 24 ±2 week follow-up | — |
Countries
India
Contacts
Public ContactDr Pragati Jain
AIIMS New Delhi
Outcome results
None listed