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Journal of scleroderma and related disorders · 2022

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

СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
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История · было → стало

Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.

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

Аннотация

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.