Artikel
Is there a difference in the presentation of diffuse and limited subtype in childhood? Results from the juvenile scleroderma inception cohort (www.juvenilescleroderma.com)
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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. Several adult publications looked at the differences between limited (ljSSc) and diffuse subtype (djSSc). There is rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort is a prospective standardized register for patients with jSSc.
Methods: Patients with jSSc were included worldwide to the jSSc cohort. We compered the demographics and clinical characteristics of the ljSSc and djSSc.
Results: Up till now 74 patients were enrolled, 54 (76%) with djSSc and 18 with ljSSc (24%). 9% in djSSc and 25% in ljSSc showed overlap features. Disease duration at time of inclusion in the cohort was 3.7 years in the djSSc and 3.3 years in ljSSc. 82% in the djSSc and 78% in the ljSSc group were female. The mean age of the onset of Raynaud symptomatic was 9,0 years in the jdSSc and 9.9 years in ljSSc group and onset of the non-Raynaud symptomatic with 9.4 in djSSc and 10.6 ljSSc.
At the time of inclusion the mean modified Rodnan Skin Score was 18.5 in the djSSc and 8,4 in ljSSc (p=0.0001). Anti-Scl 70 positivity was found in 31.5% of djSSc and 30.8% in ljSSc. Only 2 patient in the djSSc group and one in the ljSSc group was anticentromere positive. Capillary changes occurred in 60.7% in the djSSc and 50% in ljSSc, but 58.2% in djSSc and only 23.5% in ljSSc had already history of ulcerations (p=0.013) and 21.8% had active ulceration in the djSSc and 5.9% in the ljSSc. 33.3% of djSSc and 72.7% of ljSSc had cardiac involvement (p=0.027). pulmonary hypertension occurred in 57.1% djSSc and 18.2% in ljSSc. 63% in djSSc and 27.3% in ljSSc group had signs of interstitial lung disease on imaging (p=0.046). Renal involvement occurred in 7.1% djSSc and 5.6% in ljSSc. 39.3% of djSSc and 22.2% of ljSSc had gastrointestinal involvement. 56.4% in djSSc and 83.35 in ljSSc had musculoskeletal involvement (p=0.04).
Conclusion: Patients with djSSc have younger age at onset, have more often capillary changes and active ulcerations, pulmonary hypertension and less gastorintestinal and joint involvement and more disease damage. The characteristics of the pediatric subtypes differs from adults with SSc, especially the high proportion of patients with diffuse subtype.
