Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.


Update on the Juvenile Systemic Sclerosis Inception Cohort. www.juvenilescleroderma.com.

Foeldvari2,  Ivan, Wierk1,  Angela, Len8,  Claudie, Katsicas4,  Maria Martha, Tadey6,  Avcin, Kallinich7,  Till, Kone-Paut9,  Isabelll

Hamburger Zentrum Für Kinder- und Jugenrheumatologie, Hamburg, Germany
University Hospital, France
Hamburger Zentrum Fuer Kinder- und Jugendrheumatologie, Hamburg, Germany
Universitiy Childrens Hospital, Israel
Universitiy Childrens Hosptial, Brazil
Universitiy Childrens Hosptial, Austria
University Childrens Hospital, Slovenia
University Childrens Hospital, Germany
University Childrens Hospital, Brazil
University Childrens Hosptial, France

Background:

Juvenile systemic sclerosis (jSSc) is a rare autoimmune disease. Currently just retrospective data exist without a standardized assessment of the organ involvement. Our project is the first projects, where prospectively and with a standardized assessment data of early jSSc patients are collected.

Objectives:

to learn about the evolvement of juvenile systemic sclerosis

Methods:

Using the proposed standardized patient assessment protocol patients with early jSSc, entry into the cohort within the first 24 months of disease, are prospectively assessed. All participating centres approved the protocol over the own IRB.

Results:

42 centers from 20 countries applied to participate on the project. The assent and consent forms were translated into the local native languages. Up till now 16 patients were enrolled, the mean follow up of the patients in the cohort are 1.6 years. Thirteen of the 16 patients were female. The mean age at the onset of the non-Raynaud symptomatic were 12.4 years. Nine of the 16 have diffuse subtype, 5 of them have an overlap symptomatic. At the time of the inclusion the mean modified Rodnan Skin Score was 16.5 (range, 2 to 51). 14 were ANA positive, and 5 of them were anti-Scl 70 positive. None of them was anticentromere positive. Fourteen of the 16 have Raynaud`s, 12 of them have capillary changes and 4 of them already ulcerations. 7 of them have cardiopulmonary involvement, 4 of them have interstitial lung disease. Two of them have renal involvement. Eight of them have gastrointestinal involvement, and 5 of them oesophageal involvement. Fourteen of them have musculoskeletal involvement.

Conclusion:

We present the data on the first 16 prospectively assessed patients with jSSc. The current recruitment data confirms that pediatric patients are different from the adult patients. We are only at the first phase of this project and hope to recruit up to 50 patients and follow them prospectively over the next 2 to 4 years at least.

To cite this abstract, please use the following information:
Foeldvari, Ivan, Wierk, Angela, Len, Claudie, Katsicas, Maria Martha, Tadey, Avcin, Kallinich, Till, et al; Update on the Juvenile Systemic Sclerosis Inception Cohort. www.juvenilescleroderma.com. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1704
DOI: 10.1002/art.29469

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