Responsiveness of clinical measures to flare of disease activity in juvenile idiopathic arthritis.

OBJECTIVE: To compare the responsiveness of clinical measures in the assessment of disease flare in patients with juvenile idiopathic arthritis (JIA). METHODS: The clinical records of all consecutive patients with JIA who were diagnosed between 1995 and 2000 were retrospectively reviewed. In each patient, all visits made during follow-up were analyzed and those meeting the criteria for disease flare were recorded. The definition of flare was based on the therapeutic alterations made by the attending physician. Responsiveness of JIA clinical measures to relevant increase in disease activity (a flare) was evaluated by assessing the score change of each measure from a visit made 6 (+/- 3) months before a flare and the flare visit. Responsiveness statistics included the standardized response mean (SRM) and the effect size (ES). RESULTS: A total of 115 patients, who were followed for 0.5 to 6.2 years (mean 2.8 years), were studied. During follow-up, 51 patients (44%) experienced 1 or more disease flares, with the total number of flares being 75. Strong responsiveness (ES and SRM > or = 0.8) to increase in disease activity was demonstrated by the physician's and parent's global assessments, the global articular severity score, and the morning stiffness. The active, swollen and painful joint counts, the swelling, pain on motion/tenderness and limited range of motion (LROM) scores, and the erythrocyte sedimentation rate revealed moderate responsiveness (ES and SRM > or = 0.5). The poorest performances (ES and/or SRM < 0.5) were provided by the parent's assessment of pain, the functional ability tool, the number of joints with LROM, the LROM score, the C-reactive protein, the white blood cell and platelet count, and the hemoglobin level. CONCLUSION: Our analysis suggests that the swollen or painful joint counts are better suited than the count of joints with LROM for the assessment of disease flare in patients with JIA.

Influenza vaccination in children with chronic rheumatic diseases and long-term immunosuppressive therapy.

OBJECTIVE: To study the immunogenicity, safety and efficacy of influenza vaccine in children with chronic rheumatic diseases (CRD) receiving long-term immunosuppressive therapy. METHODS: Seventy children (F:M 51:19) with CRD (JIA = 49, SLE = 11, other = 10) aged 4-17 yrs and 5 healthy siblings of the patients (aged < 11 yrs) received a "split type" influenza vaccine (Fluarix SB) licensed for the 1999-2000 winter season. Clinical and laboratory evaluation were performed at study entry and at 1, 3 and 6 months after vaccination. Blood samples were collected before and one month after vaccination and antibody titers to A/Beijing, A/Sydney and B/Beijing influenza antigens were measured using a standardized hemagglutination inhibition assay. RESULTS: Patients were assigned to groups according to the therapeutic regimen [prednisone (PDN), PDN plus 1 disease modifying antirheumatic drug (DMARD), PDN plus 2 DMARDs and 1 or 2 DMARDs without PDN]. 5/70 patients reported local (3) or systemic (2) reactions and 1/5 siblings local reaction. Nine more patients reported mild upper respiratory tract symptoms 1-4 weeks post-vaccination. No patient was found to fulfill criteria for deterioration or flare of the underlying disease. At completion of vaccination 97.14% of patients developed protective HI titers to A/Beijing, 100% to A/Sydney and 80% to B/Beijing. No significant difference in the mean geometric titers was found between patients with different therapeutic regimens or age or between those with JIA or SLE. Disease activity was not related with response or non-response to B/Beijing. No patient reported "flu-like" symptoms during the 6-month period of follow-up. CONCLUSION: The results of our study indicate that children with CRD receiving long-term immunosuppressive therapy at conventional doses respond to influenza vaccination similarly to healthy children without serious adverse reactions or disease flares regardless of their age, type of CRD or therapeutic regimen.

The Greek version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report herein the results of the cross-cultural adaptation and validation into the Greek language of the parent's version of 2 health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Greek CHAQ CHQ were fully validated with 3 forward and 3 backward translations. A total of 143 subjects were enrolled: 82 patients with JIA (28% systemic onset, 24% polyarticular onset, 10% extended oligoarticular subtype, and 38% persistent oligoarticular subtype) and 61 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the systemic onset, polyarticular onset and extended oligoarticular subtypes having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Greek version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.