Article
Update on the juvenile systemic Sclerosis Inception Cohort Project. Characteristics of the first 97 Patients at first Assessment. www.juvenile-scleroderma.com
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Authors
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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
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Amra Adrovic
- Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University,, Istanbul, Turkey
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Valda Stanevicha
- University Childrens Hospital, Riga, Latvia
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Kathryn Torok
- University Childrens Hospital, Pittsburgh, United States of America
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M. T. Terreri
- Universidade Federal de São Paulo, Pediatric Rheumatology, Sao Paulo, Brasil
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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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Maria Katsicas
- Hospital de Pediatria, Buenos Aires, Argentine
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Vanessa Smith
- Gent University Hospital, Rheumatology, Gent, Belgium
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Flavio Sztajnbok
- Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brasilien
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Tadey Avcin
- University Childrens Hospital, Pediatric Rheumatology, Ljubljana, Slovenia
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Rolando Cimaz
- University of Florence, Florence, Italy
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Jordi Anton
- University Children´s Hospital, Pediatric Rheumatology, Barcelona, Spain
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Mikhail Kostik
- Saint-Petersburg State Pediatric Medical University, St. Petersburg, Russia
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Thomas Lehman
- Hospital for Special Surgery, New York, USA
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W. Alberto Sifuentes-Giraldo
- University Hospital Ramón y Cajal, Madrid, Spain
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Simone Appenzeller
- Faculty of Medical Science State University of Campinas (UNICAMP), Sao Paulo, Brazil
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Mahesh Janarthanan
- Pediatric Rheumatology, Chennai, India
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Monika Moll
- Universitätsklinikum Tübingen, Klinik für Kinder- und Jugendmedizin, Tübingen
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Dana Nemcova
- University Childrens Hospital, Pediatric Rheumatology, Prague, Czech
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Maria Jose Santos
- Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal
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Cristina Battagliotti
- hospital den Ninos Dr. Orlando Alassia, Santa Fe, Argentine
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Lillemor Berntson
- Dept. of Pediatrics, Uppsala University Hospital, Uppsala, Sweden
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Jürgen Brunner
- Medizinische Universität Innsbruck, Department für Kinder- und Jugendheilkunde, Innsbruck, Österreich
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Despina Eleftheriou
- Great Ormond Street Childrens Hospital, London, United Kingdom
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Liora Harel
- Pediatric Rheumatology, Nettnja, Israel
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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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Susan Nielsen
- Juliane Marie Centret, Rigshospitalet, Pediatric Rheumatology, Copenhagen, Danmark
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Yosef Uziel
- Pediatric Rheumatology, Sapir Medical Center, Kfar Saba, Israel
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Clarissa Pilkington
- Great Ormond Street Hospital, London, Vereinigtes Königreich
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Ann Stevens
- Seattle Children's, Seattle, USA
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Nicola Helmus
- Schön Klinik Hamburg-Eilbek, Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
| Published: | September 4, 2017 |
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Outline
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Background: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just smaller retrospective data exist regarding presentation of organ involvement. In the previous studies assessment of the organ involvement was not standardized. Our project is the first one, where data of jSSc patients were collected prospectively and with a standardized assessment. We present the data at the time of inclusion.
Methods: Patients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment.
Results: 30 centers from 19 countries agreed to participate on the project. The assent and consent forms were translated into the local native languages. Until now 97 patients have been enrolled with a mean disease duration of 3.1 years. Seventy-seven (79%) of the 97 patients were females. The mean age of the onset of Raynaud´s phenomenon was 9.6 years (+4.1 years), the youngest 1,5 years old. The mean age at the onset of the non-Raynaud presentation of jSSc was 10.3 years (+4.1 years). 68 (70%) of the 50 have diffuse subtype. 7/68 in the diffuse (10%) and 3/29 in the limited subtype (10%) had an overlap feature. Seventy-nine % of the patients received a disease modifying drug.
At the time of the inclusion the mean modified Rodnan Skin Score was 15.7, in the diffuse 18.6 and in the limited 7.7. 61/97 (63%) had already capillary changes and 44/95 (46%) already had history of ulcerations, 18/95 (19%) had active ulcerations at the time of the inclusion. 20/63 (32%) had decreased FVC under 80% and 18/39(46%) had decreased DLCO less than 80%. 28/58 (36%) had signs of interstitial lung disease on HRCT. 7/97 (7%) patients had pulmonary hypertension assessed by echo. 6/97 (6%) had urine sedimentary changes, 3 of them significant proteinuria, but no hypertension or renal crisis was observed. 28/97 (29%) of them esophageal involvement. 58/97 (59.8%) had musculoskeletal involvement. ANA positivity occurred in 77/97 (81%) and 32/92 (34%) of them were anti-Scl 70 positive. 4/62 (6%) had anticentromere positivity.
Conclusion: We present the data on the first 97 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 70% and 15% of patients with overlap features. Anti-centromere antibodies occurring only in 6% of patients.
