Artikel
Update on the juvenile systemic sclerosis inception cohort project (www.juvenilescleroderma.com). Characteristics of the first 74 patients at first assessment
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Autoren
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Ivan Foeldvari
- Schön Klinik Hamburg-Eilbek, Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
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Jens Klotsche
- Deutsches Rheuma-Forschungszentrum (DRFZ), Programmbereich Epidemiologie, Berlin
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Ozgur Kasapcopur
- Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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Amra Adrovic
- Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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M. T. Terreri
- Universidade Federal de São Paulo, Pediatric Rheumatology, Sao Paulo, Brasil
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Valda Stanevicha
- University Childrens Hospital, Riga, Latvia
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Maria Katsicas
- Hospital de Pediatria, Buenos Aires, Argentine
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Ekaterina Alexeeva
- Russian Academy of Medical Sciences, Rheumatology Department, Scientific Center for Children’s Health, Moskau, Russland
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Flavio Sztajnbok
- Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brasilien
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Rolando Cimaz
- University of Florence, Florence, Italy
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Mikhail Kostik
- Saint-Petersburg State Pediatric Medical University, St. Petersburg, Russia
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W. Alberto Sifuentes-Giraldo
- University Hospital Ramón y Cajal, Madrid, Spain
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Thomas Lehman
- Hospital for Special Surgery, New York, USA
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Dana Nemcova
- University Childrens Hospital, Pediatric Rheumatology, Prague, Czech
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Monika Moll
- Universitätsklinikum Tübingen, Klinik für Kinder- und Jugendmedizin, Tübingen
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Maria Jose Santos
- Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal
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Tadey Avcin
- University Childrens Hospital, Pediatric Rheumatology, Ljubljana, Slovenia
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Cristina Battagliotti
- hospital den Ninos Dr. Orlando Alassia, Santa Fe, Argentine
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Jürgen Brunner
- Medizinische Universität Innsbruck, Department für Kinder- und Jugendheilkunde, Innsbruck, Österreich
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Susan Nielsen
- Juliane Marie Centret, Rigshospitalet, Pediatric Rheumatology, Copenhagen, Danmark
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Tilmann Kallinich
- Charité - Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie und Immunologie, Sektion Rheumatologie, Berlin
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Kirsten Minden
- Deutsches Rheuma-Forschungszentrum (DRFZ) und Charité Universitätsmedizin Berlin, Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Berlin
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Mahesh Janarthanan
- Pediatric Rheumatology, Chennai, India
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Liora Harel
- Pediatric Rheumatology, Nettnja, Israel
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Josef Uziel
- University Children´s Hospital, Pediatric Rheumatology, Karfa Saba, Israel
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Despina Eleftheriou
- Great Ormond Street Childrens Hospital, London, United Kingdom
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Kathryn Torok
- University Childrens Hospital, Pittsburgh, United States of America
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Nicola Helmus
- Schön Klinik Hamburg-Eilbek, Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
| Veröffentlicht: | 29. August 2016 |
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Gliederung
Text
Background: 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 one, where prospectively and with a standardized assessment data of jSSc patients are collected.
Methods: Patients with jSSc 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 74 patients were enrolled. Sixty (81%) of the 74 patients were female. The mean age of the onset of Raynaud symptomatic was 9.2 years (0.2–15.9). The mean age at the onset of the non-Raynaud symptomatic were 9,7 years (0.3–15.9). 56 (76%) of the 74 have diffuse subtype, 10 (14%) of them have an overlap symptomatic.
At the time of the inclusion the mean modified Rodnan Skin Score was 16.0. ANA positive were 55/71 (77%), 24/70 (34%) of them were anti-Scl 70 positive and 3/42 (7%) was anticentromere positive. 43/74 (58%) had already capillary changes and 36/72 (50%) inactive ulcerations, 13/72 (18%) had active ulceration at the time of the inclusion. 38/74 (51%) had cardiopulmonary involvement, 19/38 (50%) of had signs of interstitial lung disease on imaging, 18/42 (43%) had FVC <80% and 12/21 (57%) had DLCO < 80%. 6/38 (16%) patients had pulmonary hypertension. 5/74 (7%) had renal involvement. 26/74 (35%) had gastrointestinal involvement, and 23/26 (88%) of them esophageal involvement. 46/73 (63%) had musculoskeletal involvement. 2/74 (3%) showed neurologic involvement.
The mean CHAQ score was 0.4 (0-2.5). Patient global disease activity on VAS (0-100) was 44.9 and disease damage 41.6. Physician global of disease activity on VAS (0-100) was 39.7 and physician global of disease damage was 34.6.
Conclusion: We present the data on the first 74 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 81%. 14% of the patients have overlap features.
