Interpretation of the Juvenile Arthritis Disease Activity Score: responsiveness, clinically important differences and levels of disease activity in prospective cohorts of patients with juvenile idiopathic arthritis.

OBJECTIVES: The objectives of this study were to assess 27-joint Juvenile Arthritis Disease Activity Score (JADAS-27) responsiveness, JADAS-27 changes corresponding to clinically important differences and cut-off scores for low and high disease activity in a large prospective JIA cohort. METHODS: JADAS-27 responsiveness, using effect size and standardized response mean (SRM), and changes in the JADAS-27 corresponding to clinically important differences were determined for clinical improvement (ACRpedi30) and worsening (flare). To assess whether various degrees of change in the JADAS-27 could be used to demonstrate improvement or worsening in individual patients, diagnostic parameters were computed for cut-off score changes. Finally, cut-off scores for low and high disease activity and their diagnostic parameters were determined. RESULTS: In 228 patients with 529 consecutive visits, ACRpedi30 was detected in 109 and flare in 111 visits. Regarding responsiveness, the effect size was 0.93 and SRM was 1.26 for clinical improvement, while for clinical worsening the effect size was 0.65 and SRM was 0.60. Changes in the JADAS-27 corresponding to clinically important difference were -5.5 for improvement and +1.7 for worsening. Cut-off score changes in the JADAS-27 had 65-90% sensitivity and 67-86% specificity for improvement, and 31-64% sensitivity and 89-97% specificity for worsening. The JADAS-27 cut-off score for low disease activity was ≤2.7 with 76% sensitivity and 62% specificity, and the cut-off score for high disease activity was ≥6 with 77% sensitivity and 77% specificity. CONCLUSION: The JADAS-27 had moderate to good responsiveness and was changed by clinically important differences. The JADAS-27 cut-off scores differentiated between low and high disease activity. These JADAS-27 interpretations could be potentially applicable in clinical care and trials.

Effects of the live attenuated measles-mumps-rubella booster vaccination on disease activity in patients with juvenile idiopathic arthritis: a randomized trial.

IMPORTANCE: The immunogenicity and the effects of live attenuated measles-mumps-rubella (MMR) vaccination on disease activity in patients with juvenile idiopathic arthritis (JIA) are matters of concern, especially in patients treated with immunocompromising therapies. OBJECTIVES: To assess whether MMR booster vaccination affects disease activity and to describe MMR booster immunogenicity in patients with JIA. DESIGN, SETTING, AND PARTICIPANTS: Randomized, multicenter, open-label clinical equivalence trial including 137 patients with JIA aged 4 to 9 years who were recruited from 5 academic hospitals in The Netherlands between May 2008 and July 2011. INTERVENTION: Patients were randomly assigned to receive MMR booster vaccination (n=68) or no vaccination (control group; n=69). Among patients taking biologics, these treatments were discontinued at 5 times their half-lives prior to vaccination. MAIN OUTCOMES AND MEASURES: Disease activity as measured by the Juvenile Arthritis Disease Activity Score (JADAS-27), ranging from 0 (no activity) to 57 (high activity). Disease activity in the year following randomization was compared between revaccinated patients and controls using a linear mixed model. A difference in JADAS-27 of 2.0 was the equivalence margin. Primary immunogenicity outcomes were seroprotection rates and MMR-specific antibody concentrations at 3 and 12 months. RESULTS: Of 137 randomized patients, 131 were analyzed in the modified intention-to-treat analysis, including 60 using methotrexate and 15 using biologics. Disease activity during complete follow-up did not differ between 63 revaccinated patients (JADAS-27, 2.8; 95% CI, 2.1-3.5) and 68 controls (JADAS-27, 2.4; 95% CI, 1.7-3.1), with a difference of 0.4 (95% CI, -0.5 to 1.2), within the equivalence margin of 2.0. At 12 months, seroprotection rates were higher in revaccinated patients vs controls (measles, 100% vs 92% [95% CI, 84%-99%]; mumps, 97% [95% CI, 95%-100%] vs 81% [95% CI, 72%-93%]; and rubella, 100% vs 94% [95% CI, 86%-100%], respectively), as were antibody concentrations against measles (1.63 vs 0.78 IU/mL; P = .03), mumps (168 vs 104 RU/mL; P = .03), and rubella (69 vs 45 IU/mL; P = .01). Methotrexate and biologics did not affect humoral responses, but low patient numbers precluded definite conclusions. CONCLUSION AND RELEVANCE: Among children with JIA who had undergone primary immunization, MMR booster vaccination compared with no booster did not result in worse JIA disease activity and was immunogenic. Larger studies are needed to assess MMR effects in patients using biologic agents. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00731965.

[A girl with a perioral rash]. / Een meisje met uitslag rond de mond.

A 5-year-old girl consulted the general practitioner with perioral skin lesions. Dermatological findings were erythema and squamae with a sharp border, without vesicles or crusts. She had a habit of licking her lips frequently, which is causal for lip licking dermatitis. After stopping this habit, the skin healed, without application of dermal therapy.