Background/Purpose: Pediatric scleroderma consists of two diseases, juvenile localized scleroderma (jLS) and juvenile systemic sclerosis (jSSc). While jLS and jSSc share some disease processes, there are major differences in their clinical features and morbidity patterns. Co-existence of LS and SSc has been reported in adults, with a recent adult systematic review reporting a frequency of 2.4-7.4%, and most patients having SSc prior to development of LS¹ To explore this issue in pediatric scleroderma, we conducted a survey over the international pediatric rheumatology email board to ask about the co-existence of jLSc and jSSc.

Methods: A 12 question was distributed over the worldwide pediatric rheumatology electronic list-serve. Data was collected in an Excel spread sheet and analyzed using Excel software.

Results: From approximately 800 survey recipients worldwide, physicians from 28 centers responded (27 pediatric rheumatologists, 1 pediatric dermatologist, half were practicing for over 20 years). Respondents followed a median of 12 jLS and 2 jSSc patients over the past 12 months, and a total of 916 jLS and 193 jSSc patients over the past 60 months. Only 7 of the patients had concurrent jLS and jSSc (0.8% of total jLS, 3.6% of total jSSc patients). Four of the patients developed jSSc following onset of jLS, with interval for 3 patients reported as 6 months, 1 year, or 6 years. Among the other 3 coexisting patients, one simultaneously presented with jLS and jSSc, and the pattern for the other two patients was not reported. jLS subtype was generalized morphea (2, one as part of mixed morphea), linear of the trunk/limb (2, one as part of mixed morphea), circumscribed superficial morphea, or mixed morphea. Many of the jLS patients who did not develop jSSc were reported to have features associated with jSSc. Raynaud Phenomenon occurred in 397 patients from 7 centers, for a collective overall frequency of 45%(879 total patients), while nailfoldcapillary changes were reported in 359 patients from 4 centers, for a collective overall frequency of 43% (842 patients). Abnormal pulmonary function or imaging tests were found in 145 from 3 centers, collective overall frequency 24%. Gut involvement including gastroesophageal reflux was found in 534 patients from 9 centers, collective overall frequency 61%.

Conclusion: In this international survey cohort of 916 jLS and 193 jSSc patients, the overall frequency of co-existence of these 2 diseases was low, only 0.8% of the jLSc patients, 3.6% of the jSSc patients. However, many jLS patients were reported to jSSc-associated symptoms including Raynaud Phenomenon. More work is needed to evaluate the features associated with co-existence of these two forms of scleroderma in children.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/how-common-is-the-coexistence-of-juvenile-localized-and-systemic-scleroderma-results-of-a-multination-survey/