PDF(Pubmed)
Abstract：
UNASSIGNED: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■比较男性和女性青少年性系统性硬化症患者的器官受累和疾病严重程度。
■人口统计，器官受累,实验室评估,我们比较了纳入前瞻性国际青少年系统性硬化症队列的男性和女性青少年发病系统性硬化症患者在基线访视时和12个月后的患者报告结局和医师评估变量.
■评估了一百七十五名青少年发病的系统性硬化症患者，142名女性和33名男性。种族,发病年龄,疾病持续时间,疾病亚型(70%弥漫性皮肤)在男性和女性之间相似。主动数字溃疡，体重指数非常低,肌腱摩擦摩擦在男性中明显更频繁。在男性中，医生对疾病严重程度和数字溃疡活动的全球评估明显更高。复合肺部受累在男性中也更常见，虽然没有统计学意义。12个月后,它们的模式差异改变女性患者肺部受累明显增多。
■在此队列中，青少年型系统性硬化症患者在基线时病程更严重,但12个月后模式发生改变.与成人调查结果的一些差异仍然存在，在男性儿科患者中没有肺动脉高压或心力衰竭信号的增加。虽然男性和女性的器官受累监测方案需要相同。
■人口统计，器官受累,实验室评估,我们比较了纳入前瞻性国际青少年系统性硬化症队列的男性和女性青少年发病系统性硬化症患者在基线访视时和12个月后的患者报告结局和医师评估变量.
■评估了一百七十五名青少年发病的系统性硬化症患者，142名女性和33名男性。种族,发病年龄,疾病持续时间,疾病亚型(70%弥漫性皮肤)在男性和女性之间相似。主动数字溃疡，体重指数非常低,肌腱摩擦摩擦在男性中明显更频繁。在男性中，医生对疾病严重程度和数字溃疡活动的全球评估明显更高。复合肺部受累在男性中也更常见，虽然没有统计学意义。12个月后,它们的模式差异改变女性患者肺部受累明显增多。
■在此队列中，青少年型系统性硬化症患者在基线时病程更严重,但12个月后模式发生改变.与成人调查结果的一些差异仍然存在，在男性儿科患者中没有肺动脉高压或心力衰竭信号的增加。虽然男性和女性的器官受累监测方案需要相同。
