Artikel
Is there a difference in the presentation of male and female patients with juvenile systemic sclerosis? 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 male and female patients with Systemic Sclerosis. There is rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort (www.juvenilescleroderma.com) is a prospective standardized register for patients with jSSc.
Methods: Patients with jSSc were included worldwide to the juvenile scleroderma inception cohort. We compered the demographics and clinical characteristics of the male and female patients.
Results: Up till now 74 patients were enrolled, 54 with djSSc (76%) and 18 with ljSSc (24%). 14 (19%) of the patients were male (M) and 60 female (F) (81%). The mean disease duration at the time of inclusion was 9.3 in M and 9.2 in F patients. 74.4% of the M and 76.7% of the F had diffuse subset. The mean age of the onset of Raynaud symptomatic was 9.3 in M and 9.2 years in the F patients and the non-Raynaud symptomatic with 9.1 in M and 9.9 in F patients. At the time of the inclusion the mean modified Rodnan Skin Score was 20 in M and 15.1 in F patients. Anti-Scl 70 positivity was found in 42.9% in M and 32.1% in F patients. Anticentromere positivity occurred in 16.7% in M and 3.3% in F patients (p=0.027). Capillary changes were present in 50% of the M and 60% of F patients, but 50% in M and F had already history of ulcerations, but 28.6% in M and 15.5% in F had active ulceration. 57.1% of the M and 50% of the F patients had cardiopulmonary involvement. Six patients had pulmonary hypertension, they were all F. 75% of M and 46.7% of F patients had signs of interstitial lung disease on imaging. Renal involvement was around 7% in both sexes. 21.4% in M and 38.3% in F patients had gastrointestinal involvement. 92.9% of M and 55.9% in M patients had musculoskeletal involvement.
Conclusion: We present the data on the first 74 patients with jSSc included in our cohort. Patients with male sex have a more severe disease similar to adult male patients.
