Background/Purpose: Juvenile systemic sclerosis (jSSc) is associated with one of the highest morbidity and mortality rate in pediatric rheumatology, yet care recommendations are based upon low quality data. As no jSSc clinical trials have been done, recommendations are primarily derived from adult trials. However, many differences in disease patterns exist between jSSc and adult SSc, including for morbidity and mortality risks, which makes this approach sub-optimal.

A key tool for conducting clinical trials are sensitive and specific classification criteria. The sensitivity of the 2007 jSSc classification criteria for classifying jSSc is < 70%, making them unfeasible to use in clinical trials. We seek to generate highly sensitive classification criteria to make it possible to conduct jSSc clinical trials. This will enable studies directed towards developing evidence-based care recommendations, and thereby improve long-term outcome for jSSc. An important first step is a comprehensive understanding of clinical features of jSSc and their frequencies. We also seek to better delineate differences between juvenile versus adult SSc.

Methods: A scoping review of jSSc and aSSc literature was conducted in PubMed and EMBASE. Search terms for jSSc and aSSc literature were determined by jSSc experts and an experienced librarian, the search was uploaded to Covidence for reviewer access. Inclusion criteria for jSSc articles were: 1) > 3 patients with jSSc as defined by authors and 2) frequency of at least some clinical features reported. Exclusion criteria were 1) Non-English articles and 2) articles of the same study population and research group. For aSSc articles literature, articles that included > 500 patients with aSSc as defined by authors and reported frequency of at least some clinical features were included. Non-English articles and articles from the same study population/group were excluded. Data extracted included disease subtype, antibody profile, and frequency of clinical features, including Raynauds phenomenon, interstitial lung disease, gastrointestinal manifestations, arthritis, sclerodactyly, tendon friction rub, heart manifestations and renal crisis.

Results: A total of 2175 articles were screened for jSSc, and 3446 for adult SSc. A higher proportion of jSSc patients had diffuse cutaneous SSc compared to adults (69.9% and 32.1%, respectively; p-value < 0.001) whereas the opposite was true for limited cutaneous SSc (20.8% and 56.9%, respectively). Arthritis was far more prominent in jSSc compared to aSSc (32.5% and 17%, respectively; p< 0.001). 8.1% of jSSc patients had positive Anti-centromere serology compared to 28.7% of aSSc patients (p-value < 0.001). 15.3% of jSSc patients had positive Anti-PM/Scl serology compared to 4.6% in aSSc patients (p-value < 0.001). Rate of renal crisis was far less in jSSc compared to aSSc patients (1.2 % vs. 3.7%, p-value=0.044).

Conclusion: Our study shows that the frequency of disease subtypes, antibody profiles and several clinical characteristics differ significantly between jSSc and aSSc patients. This supports the need for developing jSSc-specific classification criteria, and further research to better understand jSSc.

« Back to 2023 Pediatric Rheumatology Symposium

ACR Meeting Abstracts - https://acrabstracts.org/abstract/development-of-specific-classification-criteria-for-juvenile-system-sclerosis-patients-a-scoping-review/