Date: Tuesday, November 15, 2016
Session Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster III: Systemic JIA, Autoinflammatory Syndromes, Scleroderma, Vasculitis, Miscellaneous
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the retrospective studies assessment of the organ involvement is not standardized. Our project is the first project, where prospectively and with a standardized assessment data of jSSc patients are collected. We present the data of the patients at the entry into the cohort.
Methods: Patients with jSSc, according the ACR criteria, were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol.
Results: 26 centers from 17 countries applied to participate on the project. The assent and consent forms were translated into the local native languages. Up till now 80 patients were enrolled. Sixty-six (82.5%) of the 80 patients were female. The mean age of the onset of Raynaud symptomatic was 9.4 years (0.2 – 15.9). The mean age at the onset of the non-Raynaud symptomatic were 9,9 years (0.3 -15.9). 58 (72.5%) had diffuse subtype and 22 had limited subtype (27.5%). 11 (14%) of them have an overlap symptomatic, in 6 (10%) in the diffuse and 5 (23%) in limited subtype. At the time of the inclusion the mean modified Rodnan Skin Score was 15.7 (0-51). ANA positive were 60/77 (78%), 24/77 (31%) of them were anti-Scl 70 positive and 4/46 (9%) were anticentromere positive. 48/80 (60%) had already capillary changes and 36/78 (46%) inactive ulcerations, 13/78 (17%) had active ulceration at the time of the inclusion. 39/80 (49%) had cardiopulmonary involvement, 22/45 (49%) of had signs of interstitial lung disease on imaging, 16/36 (44%) had FVC <80% and 10/19 (52%) had DLCO < 80%. 6/44 (14%) patients had pulmonary hypertension. The mean 6 Minute Walk Test was 419.3 m (60-615). 5/80 (6%) had renal involvement, none of them had hypertension. 26/80 (32.5%) had gastrointestinal involvement, and 18/26 (69%) of them esophageal involvement. 48/79 (62%) had musculoskeletal involvement, mostly limited range of joint movement in 21/49 (43%). Isolated muscle weakness occurred in 3/46 (6.5%). 2/80 (2.5%) showed neurologic involvement. The mean CHAQ score (n=51) was 0.4 (0-2.5). Patient global disease activity (n=40) on VAS (0-100) was 44.4 and disease damage 41.6. Physician global of disease activity (n=44) on VAS (0-100) was 37.5 and physician global of disease damage (n=43) was 32.1.
Conclusion: We present the data on the first 80 patients with jSSc at the time of inclusion in our cohort. The current recruitment data confirms that pediatric patients are different from the adult patients; there is a significantly higher proportion of diffuse subset patients with 72.5%. 14% of the patients had an overlap features. The patients had significant disease burden assessed by patients and physician at the entry of the cohort.
To cite this abstract in AMA style:Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Stanevicha V, Sakamoto AP, Alexeeva E, Katsicas MM, Cimaz R, Kostik M, Lehman TJA, Sifuentes-Giraldo WA, Smith V, Sztajnbok F, Avcin T, Santos MJ, Nemkova D, Battagliotti C, Eleftheriou D, Harel L, Janarthanan M, Kallinich T, Anton J, Minden K, Nielsen SM, Torok KS, Uziel Y, Helmus N. Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 80 Patients at First Assessment [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/update-on-the-juvenile-systemic-sclerosis-inception-cohort-project-characteristics-of-the-first-80-patients-at-first-assessment/. Accessed June 1, 2023.
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