OBJECTIVE: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis. METHODS: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months. RESULTS: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. Composite pulmonary involvement was also more frequent in males, though not statistically significantly. After 12 months, they are the pattern of differences changed female patients had significantly more frequent pulmonary involvement. CONCLUSION: In this cohort, juvenile onset systemic sclerosis had a more severe course in males at baseline and but the pattern changed after 12 months. Some differences from adult findings persisted, there is no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in juvenile onset systemic sclerosis need to be identical for males and females.

AOU Meyer Children's Hospital Florence Italy

ASST Pini CTO Presidio Gaetano Pini Università degli Studi Milano Milan Italy

Charité University Medicine and German Rheumatism Research Center Berlin Berlin Germany

Children's Hospital at Montefiore Bronx NY USA

Department of General Paediatrics Asklepios Klinik Sankt Augustin Sankt Augustin Germany

Department of Internal Medicine Ghent University and Unit for Molecular Immunology and Inflammation VIB Inflammation Research Center Department of Rheumatology Ghent University Hospital Ghent Belgium

Department of Paediatric and Adolescents Medicine University Hospital of Cologne Cologne Germany

Department of Pediatric Rheumatology and Immunology Clinical Center Niš Faculty of Medicine University of Niš Niš Serbia

Department of Pediatric Rheumatology Cerrahpasa Medical School Istanbul University Cerrahpasa Istanbul Turkey

Department of Pediatrics and Inherited Metabolic Disorders 1st Faculty of Medicine Charles University Prague Czech Republic

Department of Pediatrics Pediatric Rheumatology Medical University Innsbruck Innsbruck Austria

Department of Women's and Children's Health Uppsala University Uppsala Sweden

Emma Children's Hospital Amsterdam University Medical Centers University of Amsterdam Amsterdam The Netherlands

German Rheumatism Research Center Berlin Germany

Great Ormond Street Hospital for Children NHS Foundation Trust London UK

Hamburg Centre for Pediatric and Adolescent Rheumatology Schön Klinik Hamburg Eilbek Hamburg Germany

Hospital de Niños Dr Orlando Alassia Santa Fe Argentina

Hospital de Pediatria J P Garrahan Buenos Aires Argentina

Hospital for Special Surgery New York NY USA

Hospital Universitário Clementino Fraga Filho Universidade Federal do Rio de Janeiro Rio de Janeiro Brazil

Jaslok Hospital and Research Center Mumbai India

Luzerner Kantonsspital Kinderspital Luzern Switzerland

National Medical Research Center of Children's Health Sechenov 1st Moscow State Medical University of the Ministry of Health of the Russian Federation Moscow Russia

Oslo University Hospital Oslo Norway

Pediatric Rheumatology Hospital Sant Joan de Déu Esplugues Universitat de Barcelona Barcelona Spain

Pediatric Rheumatology Sri Ramachandra University Chennai India

Pediatric Rheumatology Unit Meir Medical Center Kfar Saba Sackler School of Medicine Tel Aviv University Tel Aviv Israel

Pediatric Rheumatology University Tübingen Tübingen Germany

Pediatrics Department Hospital de Santa Maria Faculdade de Medicina Universidade de Lisboa Lisbon Portugal

Riga Stradins University Department of Pediatric University Children Hospital Riga Latvia

Rigshospitalet Copenhagen Denmark

Saint Petersburg State Pediatric Medical University Saint Petersburg Russia

Schneider Children's Medical Center of Israel Sackler Faculty of Medicine Tel Aviv University Petah Tikva Israel

School of Medical Science University of Campinas São Paulo Brazil

Serviço de Reumatologia Hospital Garcia de Orta Almada Portugal

Sir Ganga Ram Hospital New Delhi India

Stony Brook Children's Hospital Stony Brook NY USA

The Hospital for Sick Children University of Toronto Toronto ON Canada

Toronto Scleroderma Program Toronto Western Hospital Mount Sinai Hospital University of Toronto Toronto ON Canada

Universidade do Estado Rio de Janeiro Brazil

Universidade Federal de São Paulo São Paulo Brazil

University Children's Hospital University Medical Center Ljubljana Ljubljana Slovenia

University Hospital Ramón y Cajal Madrid Spain

University of Missouri Columbia Columbia MO USA

University of Pittsburgh Children's Hospital of Pittsburgh Pittsburgh PA USA

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