Date: Sunday, November 8, 2015
Session Type: ACR Poster Session A
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 previous retrospective studies assessment of the organ involvement was not standardized. Our project is the first one, where data of jSSc patientes were collected prospectively and with a standardized assessment.
Methods: Patients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol. Data of the juvenile systemic sclerosis inception cohort have been contributed to the DeSScipher project which was funded by a grant of the European Community’s Framework Programme 7 under grant agreement N° 305495.”
24 centers from 16 countries agreed to participate on the project. The assent and consent forms were translated into the local native languages. Until now 50 patients have been enrolled with a mean disease duration of 6.1 years. Thirtyseven (74%) of the 50 patients were females. The mean age of the onset of Raynaud´s phenomenon was 9.2 years (2-16 years), the youngest 2 years old. The mean age at the onset of the non-Raynaud presentation of jSSc was 9.7 years (2.3-16.00 years). 34 (68%) of the 50 have diffuse subtype. 5 in the diffuse (14.7%) and 3 in the limited subtype (19%) had an overlap feature.
At the time of the inclusion the mean modified Rodnan Skin Score was 16.7. 32/48 (67%) had already capillary changes and 25/48 (52%) already had history of ulcerations, 12/48 (25%) had active ulcerations at the time of the inclusion. 35/50 (70%) had cardiopulmonary involvement, 18/50 (36%) had signs of interstitial lung disease. 5 (10%) patients had pulmonary hypertension. Four (8%) had renal involvement, but no renal crisis. 20/50 (40%) had gastrointestinal involvement and 15 (30%) of them esophageal involvement. 38/49 (77.5%) had musculoskeletal involvement. ANA positivity occurred in 36/47 (77%) and 15/40 (37.5%) of them were anti-Scl 70 positive. 2/29 (7%) had anticentromere positivity.
Conclusion: We present the data on the first 50 patients with jSSc included in our cohort. The current recruitment data confirms that pediatric patients are different from the adult patients, with a higher proportion of diffuse subset patients with 68% and 16% of patients with overlap features. Anti-centromere antibodies occuring only in 7% of patients.
To cite this abstract in AMA style:Foeldvari I, Klotsche J, Staņeviča V, Alexeeva E, Katsicas MM, Terreri MT, Sakamoto AP, Cimaz R, Kostik M, Avcin T, Santos MJ, Moll M, Nemkova D, Sztajnbok F, Battagliotti C, Brunner J, Eleftheriou D, Sifuentes Giraldo A, Harel L, Janarthanan M, Kallinich T, Minden K, Nielsen SM, Torok KS, Uziel Y, Helmus N. Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 50 Patients at First Assessment. Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/update-on-the-juvenile-systemic-sclerosis-inception-cohort-project-characteristics-of-the-first-50-patients-at-first-assessment-www-juvenile-scleroderma-com/. Accessed September 25, 2021.
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