Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort

Ivan Foeldvari*, Jens Klotsche, Ozgur Kasapcopur, Amra Adrovic, Maria Teresa Terreri, Ana Paula Sakamoto, Valda Stanevicha, Jordi Anton, Brian M Feldman, Flavio Sztajnbok, Raju Khubchandani, Ekaterina Alexeeva, Maria Katsicas, Sujata Sawhney, Vanessa Smith, Simone Appenzeller, Tadej Avcin, Mikhail Kostik, Thomas Lehman, Edoardo MarraniDieneke Schonenberg-Meinema, Walter-Alberto Sifuentes-Giraldo, Natalia Vasquez-Canizares, Mahesh Janarthanan, Monika Moll, Dana Nemcova, Anjali Patwardhan, Maria Jose Santos, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Jürgen Brunner, Rolando Cimaz, Patricia Costa-Reis, Despina Eleftheriou, Liora Harel, Gerd Horneff, Sindhu R Johnson, Daniela Kaiser, Tilmann Kallinich, Dragana Lazarevic, Kirsten Minden, Susan Nielsen, Farzana Nuruzzaman, Siri Opsahl Hetlevik, Yosef Uziel, Nicola Helmus, Kathryn S Torok

*Corresponding author af dette arbejde

Abstract

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.

OriginalsprogEngelsk
TidsskriftJournal of scleroderma and related disorders
Vol/bind8
Udgave nummer2
Sider (fra-til)120-130
Antal sider11
ISSN2397-1983
DOI
StatusUdgivet - jun. 2023

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