Symptomatic polyautoimmunity at diagnosis of 1463 childhood-onset lupus: A Brazilian multicenter study

Abstract

Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome.

Autoimmune thyroid diseases, autoimmune hepatitis, celiac disease and type 1 diabetes mellitus in pediatric systemic lupus erythematosus: Results from the CARRA Legacy Registry

“…We used a strict definition of PA that did not include APS and SS, which were reported in adult studies to be the most frequent autoimmune disorders associated with lupus. 5,16 However, the prevalence we reported was similar to other pediatric studies whether included APS and SS (9.8% in Setoue, 2018) 6 or excluded APS and SS (9.7% in Aikawa, 2012). 4…”

“…Finally, our estimates and data could be overestimations given that we had PA data on 30% of the cohort; however, our prevalence estimates of PA were similar to a large Brazilian-pediatric cohort. 6 Our next step is to validate our results using the new CARRA (CARRA II) Registry which has a prospective, longitudinal data collection. The new CARRA database will provide more complete data on other autoimmune disorders which will lead to incidence estimation and collects more details on lupus disease outcomes including SDI score for disease damage.…”

“…7–10 In a cohort of 1463 children with lupus, Setoue et al. 6 described patients with symptomatic PA (including APS 2.8% and SS 0.07%) at the time of lupus diagnosis. Authors found that patients with symptomatic PA had less prevalent renal involvement but more APS; however, SLEDAI-2K scores were not different.…”

“…4–6 Studies describing the association of PA with severe lupus primarily focused on disease activity and/or damage and overlooked patient’s quality of life as an important outcome domain. 2,3,6–10 Although PA in pediatric lupus population is described, few studies have addressed its relation to lupus outcomes. 3,6,10…”

“…2,3,6–10 Although PA in pediatric lupus population is described, few studies have addressed its relation to lupus outcomes. 3,6,10…”