MANCHESTER, England -- Mortality remains high among young patients with juvenile idiopathic arthritis (JIA), and particularly for those with the severe, systemic subtype, a review of data from the British Society for Pediatric and Adolescent Rheumatology (BSPAR) Etanercept Cohort Study showed.
The standardized mortality ratio for death among all JIA patients was 7.3 (95% CI 2.9-15), according to , MBBS, of the University of Manchester, and colleagues.
But for those with severe systemic JIA -- defined as requiring treatment with biologics -- the standardized mortality ratio was 21.7 (95% CI 5.9-55.4) compared with 3.9 (95% CI 0.8-11.3) among patients with nonsystemic disease, she reported during a poster session at the here. The mortality ratios compared death rates among JIA patients in these categories with the age- and gender-matched general U.K. population.
"These data highlight just how severe the disease can be, which not everyone realizes," Davies told MedPage Today.
"It's important to have these numbers, with new treatments including biologics and stem-cell transplants being more widely used," she said.
Mortality rates among patients with JIA are higher than rates in the general population, and rates in systemic JIA are expected to be higher than among other subtypes of JIA, but data have thus far been lacking.
To address this gap in knowledge, Davies identified deaths in BSPAR among children being treated with etanercept (Enbrel) through regular follow-up from registration through December 2012.
The JIA cohort included 693 patients, 99 of whom had the systemic subtype. Their mean age at the time of study entry was 11 years, and median disease duration was 4 years.
A total of seven deaths occurred during follow-up, four of which were in the systemic group, and all deaths were in females.
Causes of death included complications of systemic JIA following a stem-cell transplant in four, and one each from asthma, infected joint prosthesis, and macrophage activation syndrome.
The mortality incidence rate per 1,000 patient-years was 1.6 (95% CI 0.6-3.2) overall. For systemic JIA, the incidence rate per 1,000 was 5 (95% CI 1.4-12.9), while for other subtypes of JIA, the rate per 1,000 was 0.8 (95% CI 0.2-2.4).
For females, the mortality incidence rate overall was 2 per 1,000 (95% CI 0.7-4.4), while rates were 8.6 per 1,000 (95% CI 2.3-22) for the systemic subtype and 0.8 (95% CI 0.1-2.4) for other subtypes.
The standardized mortality ratio for females overall was 11.9 (95% CI 4.4-25.9) and 4.7 (95% CI 0.6-17) for nonsystemic subtypes but 49.5 (95% CI 13.5-126.8) for the systemic subtype.
Disclosures
The authors reported no financial conflicts of interest.
Primary Source
British Society for Rheumatology
Source Reference: Davies R, et al "Standardized mortality rates are increased in patients with severe juvenile idiopathic arthritis" BSR 2015; Abstract 266.