Date: Sunday, November 8, 2020
Session Type: Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. Longitudinal prospective follow up data of patients with jSSc is rare. In the international juvenile systemic scleroderma cohort (jSScC) patients are followed with a standardized assessment prospectively.
Methods: Patients diagnosed according the ACR 2013 criteria for systemic sclerosis were included, if they developed the first non-Raynaud symptom before the age of 16 and were under the age of 18 at the time of inclusion. Patients were followed prospectively every 6 months with a standardized assessment.
Results: 39 patients in the JSScC had 36 months follow up. 80% had a diffuse subtype. 95% of the patients were Caucasian origin and 80% female. Mean disease duration at time of inclusion was 3.5 years. Mean age onset of Raynaud’s was 8.8 years and mean age of onset at the first non-Raynaud´s was 9.5 years. The MRSS dropped from the time point of the inclusion into the cohort from 13.9 to 11.8 after 36 months. Pattern of organ involvement did not show any significant change, beside the increase of the nailfold capillary changes from 49% to 73% (p=0.037). No renal crisis occurred. No mortality was observed.
They were positive significant changes in the patient related outcomes assessed on a VAS scale of 0 to 100. The physician global disease activity decreased from 40.0 to 22.1 (p < 0.001).
Patients global disease activity decreased from 43.3 to 20.4 and patients global disease damage from 45.0 to 21.7 (p< 0.001).
Conclusion: After 36 months follow up, we could observe a significant improvement of patient related outcomes and only one significant change in organ pattern involvement. In a mostly diffuse subset patient population this is a very promising result regarding outcome.
Supported by the “Joachim Herz Stiftung”
To cite this abstract in AMA style:Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Terreri M, Marrani E, Avcin T, Katsicas M, Nemcova D, Santos M, Brunner J, Kallinich T, Kostik M, Minden K, Patwardhan A, Torok K, Helmus N. No Disease Progression After 36 Months Follow up in the Juvenile Systemic Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/no-disease-progression-after-36-months-follow-up-in-the-juvenile-systemic-scleroderma-inception-cohort/. Accessed July 30, 2021.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/no-disease-progression-after-36-months-follow-up-in-the-juvenile-systemic-scleroderma-inception-cohort/