Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.
Therapeutic Approaches for the Treatment of Active Juvenile Dermatomyositis: An International Multicenter Study.
Hasija3, Rachana, Pistorio3, Angela, Ravelli3, Angelo, Demirkaya3, Erkan, Khubchandani3, Raju, Guseinova3, Dinara, Malattia3, Clara
Recently published articles have documented a marked improvement in long-term outcome and survival of juvenile dermatomyositis (JDM) patients but little information is available on standardized evaluations of response to therapy based on current treatment options.
Our aim was to evaluate in large prospective cohort of JDM patients the response to therapy over a 24 months period, according to the PRINTO JDM response criteria.
Patients & Methods:
Clinical, laboratory and therapeutic modalities were collected prospectively between 2001 and 2004 in JDM patients by PRINTO/PRCSG members from 36 countries. Patients with probable or definite JDM, age < 18 years, in an active phase of their disease, at 4 time points (baseline, 6, 12 and 24 months), were included. The validated core set variables were the global assessment by the physician and parent, muscle strength, functional ability, quality of life and disease activity tool. Patients were defined as improved if able to demonstrate at least 20% (50, 70, or 90) improvement from baseline in 3 of any 6 core set variables with no more than 1 of the remaining worsening by more than 30%, which cannot be muscle strength. Remission was defined as patients with normal muscle strength (CMAS >=48; nv 052) and physician global assessment of disease activity <= 0.5 cm (nv 010) and normal CPK (<=150 U/L)
The analysis data set included 275/294 (94%) patients. Patients median ages at onset and disease duration visit were 7.2 and 6 months respectively with 168 (61%) being female. The greatest improvement in clinical and laboratory measures was observed in the first 6 months of therapy and maintained thereafter. At baseline treatment options included steroids in 269 (97.8%), metotrexate (MTX) in 134 (48.7%) with 91 newly started, cyclosporine A in 44 (16%), hydroxychloroquine in 37 (13.5%), and IVIG in 38 (13.8%). Oral steroids dose at baseline and 6 months were 1 and 0.3 mg/kg/day respectively, at 24 months 91 patients (52.3%) were still on steroids at 0.21 mg/kg/day; steroids pulses were used in 100 (36.4%) at baseline. Figure reports the 20, 50, 70, 90 response and remission over time.
Figure 1. PRINTO JDM PDI 20, 50, 70, 90 and remission rate in 275 patients at 6, 12 and 24 months.
A substantial improvement was observed in the initial 6 months (PRINTO 20 criteria 84%) and maintained thereafter.
Six months of therapy lead to a significant improvement in JDM core set measures and response criteria, that was maintained up to 2 years follow-ups. These data provided standardized response to therapy data in patients with JDM treated according to the current available options.
To cite this abstract, please use the following information:
Hasija, Rachana, Pistorio, Angela, Ravelli, Angelo, Demirkaya, Erkan, Khubchandani, Raju, Guseinova, Dinara, et al; Therapeutic Approaches for the Treatment of Active Juvenile Dermatomyositis: An International Multicenter Study. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1700