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拉脱维亚系统性硬化症队列的流行率和性别特异性分析。

Prevalence and gender - specific analysis of a systemic sclerosis cohort in Latvia.

机构信息

Department of Doctoral Studies, Rīga Stradinš University, Riga, Latvia.

Institute of Oncology and Molecular Genetics, Rīga Stradinš University, Riga, Latvia.

出版信息

Orphanet J Rare Dis. 2024 Sep 30;19(1):361. doi: 10.1186/s13023-024-03355-y.

DOI:10.1186/s13023-024-03355-y
PMID:39350191
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11443687/
Abstract

BACKGROUND

Systemic sclerosis (SSc) is considered by many to be one of the most severe autoimmune rheumatic diseases with lower prevalence observed in Northern Europe. No previous studies on the prevalence of SSc in Latvia have been conducted and the aim was to study the demographic and clinical data of patients with SSc in northeastern Europe country.

METHODS

This study was conducted in two main Latvian hospitals for adults and includes patients with SSc who were consulted between 2016 and 2021.

RESULTS

During the study period, 159 patients with SSc were consulted. The point prevalence on 1 January 2021 was 84.0 per million. Female to male ratio was 4.67:1, and highest gender ratio was observed in the age group 70-79-year (6.75:1). Antinuclear antibodies were present in 82.58% of patients, without gender difference. Centromere pattern was more frequently observed in females (40.19% vs. 19.04%), in contrast to speckled pattern (50.98% vs. 57.14%). At disease onset females tended to be younger (46.51 ± 13.52) than males (50.5 ± 16.64). Males had more diffuse cutaneous subtype, interstitial lung disease, pulmonary hypertension and esophageal dysmotility. More than half of patients received treatment with glucocorticoids at any point of the disease (68.31%), without gender difference.

CONCLUSIONS

Systemic sclerosis is less common in Latvia than in other countries and regions. Due to its location, the data from Latvia are consistent with a north-south gradient in Europe. Gender ratio differences persisted in older age groups as well. Antinuclear antibodies presence did not differ between genders, but in female's centromere pattern was much more likely to be present. Males had more severe disease course, but in both genders more than half of patients received treatment with GCs at any point of the disease.

摘要

背景

系统性硬化症(SSc)被许多人认为是最严重的自身免疫性风湿病之一,在北欧的发病率较低。以前在拉脱维亚没有关于 SSc 患病率的研究,本研究旨在研究东欧国家拉脱维亚 SSc 患者的人口统计学和临床数据。

方法

该研究在拉脱维亚的两家主要成人医院进行,包括 2016 年至 2021 年间就诊的 SSc 患者。

结果

在研究期间,共咨询了 159 例 SSc 患者。2021 年 1 月 1 日的时点患病率为 84.0/百万人。男女比例为 4.67:1,70-79 岁年龄组的性别比最高(6.75:1)。抗核抗体在 82.58%的患者中存在,无性别差异。着丝粒模式在女性中更为常见(40.19%比 19.04%),而斑点模式在女性中更为常见(50.98%比 57.14%)。在疾病发病时,女性比男性更年轻(46.51±13.52 岁比 50.5±16.64 岁)。男性更易发生弥漫性皮肤型、间质性肺病、肺动脉高压和食管动力障碍。超过一半的患者在疾病的任何阶段都接受过糖皮质激素治疗(68.31%),无性别差异。

结论

系统性硬化症在拉脱维亚的发病率低于其他国家和地区。由于其地理位置,拉脱维亚的数据与欧洲的南北梯度一致。性别比差异在年龄较大的年龄组中仍然存在。抗核抗体的存在在性别之间没有差异,但在女性中着丝粒模式更常见。男性的疾病病程更严重,但在两性中,超过一半的患者在疾病的任何阶段都接受过 GCs 治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3250/11443687/c115eb7a2670/13023_2024_3355_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3250/11443687/21740820d7b7/13023_2024_3355_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3250/11443687/c115eb7a2670/13023_2024_3355_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3250/11443687/21740820d7b7/13023_2024_3355_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3250/11443687/c115eb7a2670/13023_2024_3355_Fig2_HTML.jpg

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