Article Text
Abstract
Background Systemic juvenile arthritis (Still's disease, ICD-10 M08.2) is a severe condition, persisting in the majority of cases into adulthood.
Objectives To assess the outcomes of systemic juvenile arthritis in adult patients.
Methods Analysis of personal database for the period of 12-year follow up (2003-2014 years). Therapy, disease activity (DAS28), radiographic stage (Steinbrocker criteria), functional class (Steinbrocker criteria), bone avascular necrosis status and nanism, as well as social status (education, professional activity, marital status) of the patients were analyzed.
Results The data base contains data on 29 patients with Still's disease with the onset at 6.1±4.2 years: 18 females, 11 males aged 17-29 years, with disease duration of 6-25 years. Glucocorticoids were prescribed to all patients (dose 10-70 mg/day, therapy duration 1-23 years), 18 - continue taking them. Disease-modifying anti-rheumatic drugs were also prescribed to all patients: methotrexate – 24 (dose 7.5-30 mg/week, duration 1-17 years), leflunomide – 15, cyclosporine – 10, hydroxychloroquine – 7, azathioprine – 6, sulfasalazine – 4, D-penicillamine – 3. Biological agents (BA) were administered in 14 patients (including 1 BA – to 5 patients, 2 BAs – 7, 3 BAs – 2 patients): infliximab – 10, tocilizumab – 5, rituximab – 4, etanercept – 4, abatacept – 1, adalimumab – 1. Active disease was established in 72.4% patients, medically induced remission (≥2 years) – in 5, non-medical remission (≥2 years) – in 3 patients. I radiographic stage was found in 1, II – in 11, III – in 8, IV – in 9 patients. I functional class was detected in 1 patient, II – in 24, III – in 3 patients, one patient was wheel-chaired. Avascular bone necrosis was diagnosed in 55.2% patients, nanism – in one out of 3 patients. Higher education has 10 patients, vocational secondary education – 17 patients, secondary education - 2 patients. Out of 13 graduated patients 11 have work, and 2 depend on the family. 12 have their own families, 2 patients have children.
Conclusions Presented from long-term follow up data on adulthood outcomes in patients with systemic juvenile arthritis (Still's disease) indicate, that this rare entity is of medical and social importance, requiring further studies.
References
Nigrovic P.A., White P.H. Care of the adult with juvenile rheumatoid arthritis. Arthritis Rheum., 2006, 55, 2, 208-216.
Coulson E.J., Hanson H.J.M., Foster H.E. What does an adult rheumatologist need to know about juvenile idiopathic arthritis? Rheumatology, 2014, 53, 2155-2166.
Disclosure of Interest None declared