Author: Jessica Perfetto
Matt et al. (0363) assessed clinical, inflammatory, and pulmonary outcomes of post-allogenic hematopoietic stem cell transplant (HSCT) in an international cohort of children with refractory systemic JIA (sJIA), recurrent macrophage activation syndrome (MAS), and sJIA-related lung disease (sJIA-LD). Six months post-HSCT, 80% (10/13) patients showed complete clinical response (no active features of sJIA or MAS) without biologics and 78% (7/9) with sJIA-LD demonstrated improvement; 15% (2/13) died due to infection and/or graft-versus-host disease.
Nowicki et al. (2053) assessed pre-treatment clinical variables associated with response to NSAID monotherapy versus addition of second-line therapy in children with chronic non-bacterial osteomyelitis (CNO) at a single centre. Children with 2 or more regions affected by CNO were 1.94 times more likely to require second-line treatment compared to NSAID monotherapy (p=0.01), and those with symmetric lesions were 6.86 times more likely to require second-line treatment (p<0.001).
Maccora et al. (0373) identified potential tear-based inflammatory biomarkers of uveitis in children with JIA using advanced proteomic strategies. 13 proteins were differentially expressed in JIA patients with and without uveitis (8 statistically significantly different). There were more differentially expressed proteins between uveitis and no uveitis in children with inactive arthritis.
Stern et al. (1245) evaluated the sensitivity of the CARRA Sjogren’s Workgroup clinical diagnostic algorithm for childhood Sjogren’s disease (cSjD) in an international cohort of 300 children. 100 (33%) children with cSjD were positive based on the algorithm. Of the 100 (33%) children with complete data, 75 (75%) were positive across all pathways. Within each of the 4 pathways, sensitivity ranged from 23-42% (all 300 cSjD cases) and 50-82% (limited to the 100 [33%] cSjD cases with complete data).
Ringold et al. (0831) assessed CARRA STOP-JIA Consensus Treatment Plans (CTPs) in children with untreated polyarticular JIA (pJIA) (1. Step-Up (SU), 2. Early Combination (EC), 3. Biologic First (BF)). The primary outcome was % of children achieving ACR clinically inactive disease (CID) off glucocorticoids at 2 and 3 years. In the 325 children, CID at 2 years was 42% for SU, 58% EC, and 52% for BF (p=0.03 for SU vs EC), but there were no statistically significant differences at 3 years. However, EC was significantly better than SU for outcomes reflecting duration of time spent with less disease activity.
Glaser et al. (1621) aimed to characterise and quantify the incidence of non-central nervous system (CNS) extracutaneous manifestations (ECMs) in children with craniofacial scleroderma using maxillofacial MRI. In the 37 patients, there was a high prevalence of non-CNS ECMs: bone (49%), soft tissue (73%), adipose (57%), gland (57%), muscle (74%). Clinical exam underestimated radiographic changes in the upper (76% vs 84%) and lower (65% vs 78%) facial thirds.
Soulsby et al. (1624) explored the association of race with achievement of low lupus disease activity state (LLDAS) in children with paediatric SLE in the CARRA Registry. In a racially diverse cohort of 540 children, children of Black race and more than one race were less likely to achieve LLDAS, despite adjustment for social determinants of health (SDoH) and disease characteristics (adjusted OR 0.56, 95% CI: 0.38-0.82; OR 0.51, 95% CI: 0.31-0.86, respectively).
ABOUT THE AUTHOR
Jessica Perfetto
Jessica is a 3rd year paediatric rheumatology fellow at The Children’s Hospital at Montefiore in the Bronx, New York. She is interested in JDM and global health.
Outside of work, she loves being outdoors and active – swimming, biking, hiking, and long walks in the city. She also loves traveling and salsa dancing.
Jessica is the country representative for the US of PReS EMERGE.