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
Hamburg Centre for Pediatric and Adolescent Rheumatology, Schön Klinik Hamburg Eilbek, Hamburg, Germany.
German Rheumatism Research Center, Berlin, Germany.
J Scleroderma Relat Disord. 2023 Jun;8(2):120-130. doi: 10.1177/23971983221143244. Epub 2022 Dec 19.
To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis.
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.
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.
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.
比较青少年起病的系统性硬化症男性和女性患者的器官受累情况及疾病严重程度。
对前瞻性国际青少年系统性硬化症队列中入组的青少年起病的系统性硬化症男性和女性患者，在基线访视时及12个月后，比较其人口统计学特征、器官受累情况、实验室评估、患者报告结局及医生评估变量。
共评估了175例青少年起病的系统性硬化症患者，其中女性142例，男性33例。男性和女性在种族、起病年龄、病程及疾病亚型（70%为弥漫性皮肤型）方面相似。男性患者中活动性指端溃疡、极低体重指数及肌腱摩擦音更为常见。男性患者的医生对疾病严重程度及指端溃疡活动度的整体评估显著更高。男性患者合并肺部受累也更常见，尽管无统计学意义。12个月后，差异模式发生变化，女性患者肺部受累更为频繁。
在该队列中，青少年起病的系统性硬化症男性患者在基线时病程更为严重，但12个月后模式发生改变。与成人研究结果的一些差异仍然存在，男性儿童患者中肺动脉高压或心力衰竭的信号未增加。青少年起病的系统性硬化症器官受累监测方案对男性和女性应相同。
