TY - JOUR
T1 - Gender differences in juvenile systemic sclerosis patients
T2 - Results from the international juvenile scleroderma inception cohort
AU - Foeldvari, Ivan
AU - Klotsche, Jens
AU - Kasapcopur, Ozgur
AU - Adrovic, Amra
AU - Terreri, Maria Teresa
AU - Sakamoto, Ana Paula
AU - Stanevicha, Valda
AU - Anton, Jordi
AU - Feldman, Brian M.
AU - Sztajnbok, Flavio
AU - Khubchandani, Raju
AU - Alexeeva, Ekaterina
AU - Katsicas, Maria
AU - Sawhney, Sujata
AU - Smith, Vanessa
AU - Appenzeller, Simone
AU - Avcin, Tadej
AU - Kostik, Mikhail
AU - Lehman, Thomas
AU - Marrani, Edoardo
AU - Schonenberg-Meinema, Dieneke
AU - Sifuentes-Giraldo, Walter Alberto
AU - Vasquez-Canizares, Natalia
AU - Janarthanan, Mahesh
AU - Moll, Monika
AU - Nemcova, Dana
AU - Patwardhan, Anjali
AU - Santos, Maria Jose
AU - Battagliotti, Cristina
AU - Berntson, Lillemor
AU - Bica, Blanca
AU - Brunner, Jürgen
AU - Cimaz, Rolando
AU - Costa-Reis, Patricia
AU - Eleftheriou, Despina
AU - Harel, Liora
AU - Horneff, Gerd
AU - Johnson, Sindhu R.
AU - Kaiser, Daniela
AU - Kallinich, Tilmann
AU - Lazarevic, Dragana
AU - Minden, Kirsten
AU - Nielsen, Susan
AU - Nuruzzaman, Farzana
AU - Opsahl Hetlevik, Siri
AU - Uziel, Yosef
AU - Helmus, Nicola
AU - Torok, Kathryn S.
N1 - Funding Information:
The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: The inception cohort project was supported by an unrestricted grant from the Joachim Hertz Stiftung, Hamburg, Germany.
Publisher Copyright:
© The Author(s) 2022.
PY - 2023/6
Y1 - 2023/6
N2 - 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.
AB - 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.
KW - clinical characteristics
KW - disease severity
KW - gender
KW - juvenile systemic sclerosis
KW - male
KW - Scleroderma
UR - http://www.scopus.com/inward/record.url?scp=85144306905&partnerID=8YFLogxK
U2 - 10.1177/23971983221143244
DO - 10.1177/23971983221143244
M3 - Article
C2 - 37287945
AN - SCOPUS:85144306905
SN - 2397-1983
VL - 8
SP - 120
EP - 130
JO - Journal of Scleroderma and Related Disorders
JF - Journal of Scleroderma and Related Disorders
IS - 2
ER -