Article
Is there a difference in presentation of female and male patients with juvenile systemic scleroderma?
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Authors
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Ivan Foeldvari
- 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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Kathryn Torok
- University Childrens Hospital, Pittsburgh, United States of America
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Valda Stanevicha
- University Childrens Hospital, Riga, Latvia
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Flavio Sztajnbok
- Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brasilien
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Ana Paula Sakamoto
- Universidade Federal de São Paulo, Sao Paulo, Brazil
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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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Jordi Anton
- University Children’s Hospital, Pediatric Rheumatology, Barcelona, Spain
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Maria Katsicas
- Hospital de Pediatria, Buenos Aires, Argentina
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Vanessa Smith
- Gent University Hospital, Rheumatology, Gent, Belgium
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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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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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Amara Nassar
- Amsterdam UMC, Amsterdam, Netherland
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Cristina Battagliotti
- Hospital den Ninos Dr. Orlando Alassia, Santa Fe, Argentina
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Lillemor Berntson
- Dept. of Pediatrics, Uppsala University Hospital, Uppsala, Sweden
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Blanca Bica
- Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil
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Jürgen Brunner
- Medizinische Universität Innsbruck, Department für Kinder- und Jugendheilkunde, Innsbruck, Österreich
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Patrícia Costa Reis
- Hospital de Santa Maria, Lisbon, Portugal
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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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Gerd Horneff
- Asklepios Klinik Sankt Augustin, Sankt Augustin
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Tilmann Kallinich
- Charité - Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie und Immunologie, Sektion Rheumatologie, Berlin
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Dragana Lazarevic
- KC Niš, Nis, Serbia
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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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Farzana Nuruzzaman
- Stony Brook Children’s Hospital, Stony Brook, NY, USA
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Anjali Patwardhan
- University of Missouri, Columbia, USA
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Yosef Uziel
- Pediatric Rheumatology, Sapir Medical Center, Kfar Saba, Israel
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Nicola Helmus
- Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
| Published: | October 8, 2019 |
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Outline
Text
Background: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. There are limited data published regarding the differences in clinical presentation of male and female patients with jSSc. The Juvenile Systemic Scleroderma Inception Cohort (JSSIC) is a multinational cohort with a prospective standardized assessment of the patients. The data regarding the difference in clinical characteristics at time of inclusion in the cohort are presented.
Methods: The JSSIC is a prospective multicentre registry of patients with jSSc, who fulfil the adult classification criteria, and presented the first non-Raynaud symptoms before 16 years old and were younger than 18 years old at the time of inclusion in the cohort. Patients characteristics at time of inclusion in the cohort were evaluated.
Results: As of 15th of December 2018 120 patients are included in JSSIC. The great majority are female (80%). There were more female patients with CK elevation (29% vs 22%) and more female patients with Gottron papulae (25% vs 12%). The mean modified skin score was higher in males (18.6 vs 13.9).
Sclerodactyly was more frequent in males (90% vs 76%). Active ulceration was present in 33% of males compared to 14% of females (p=0.026). FVC<80% occurred more often in males with 47% compared with 24% in females (p=0.018). Pulmonary hypertension was more common in females with 7% compared to 4% in males. Urine sediment changes were more common in males (8% vs 4%). Gastrointestinal involvement was more common in females (37% vs 29%). Contractures occurred more often in males with 62% compared with 46% in females. Tendon friction rub was observed in 21% of males and 3% of females (p=0.001). Physician global scores of disease activity and damage were higher in males with 48 for both assessments compared to 36 and 30 in females.
Conclusion: Male patients with jSSc have a higher severity of disease, as it has been reported in adults.
Supported by the "Joachim Herz Stiftung"
