Clinical characteristics and outcomes of 566 Thais with systemic sclerosis: A cohort study
Clinical characteristics and outcomes of 566 Thais with systemic sclerosis: A cohort study
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DOI：
10.1111/1756-185x.13859
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发表时间：
2020-05-18
影响因子：
2.5
通讯作者：
Nanagara, Ratanavadee
中科院分区：
文献类型：
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作者：
Foocharoen, Chingching;Peansukwech, Udomlack;Nanagara, Ratanavadee
背景：与大多数高加索人和一些亚洲人不同，大多数泰国系统性硬化症(SSC)患者患有弥漫性皮肤硬化症(DcSSc)。目的了解泰国局限性皮肤SSc(LcSSc)与局限性皮肤SSc(DcSSc)的临床差异，明确其死亡率及相关因素。方法对2013年1月至2019年6月间566例泰国成年SSc患者进行队列研究。结果女性多于男性(356例比210例)。本组病例以直肠癌为主(411例，72.6%)。肺纤维化(PF)检测时的中位病程为2.5年，肺动脉高压为8.1年，肾危象为4.1年。通过GEE分析，dcSSc与盐和胡椒皮肤、手畸形以及改良Rodnan皮肤评分(MRSS)每增加1分显著相关。发病年龄60岁(危险比[HR]5.5)、世界卫生组织功能分级(FC)III级(HR 5.1)、FC IV级(HR 34.8)、皮肤浮肿(HR 11.4)、PF起病早(HR 1.7)、MRSS评分每增加5分(HR 4.5)、有2个脏器受累(HR 10.1)与较大的死亡风险相关。Pf是SSC的早期并发症，Pf检测越早预后越差。发病年龄大、高FC、皮肤严重紧绷和多器官受累与更大的死亡风险相关。
Background Most Thai patients with systemic sclerosis (SSc) have diffuse cutaneous SSc (dcSSc) unlike most Caucasians and some Asians. A longitudinal cohort study among Thai dcSSc is needed.Objectives We aimed to determine the overall clinical characteristics, define the clinical difference between limited cutaneous SSc (lcSSc) and dcSSc, and ascertain the mortality rate and the factors associated with mortality.Method We conducted a cohort study including 566 Thai adult SSc patients between January 2013 and June 2019. Clinical difference between lcSSc and dcSSc was investigated using generalized estimating equations (GEE).Results Females presented more than males (356 vs 210 cases). The majority of cases were dcSSc (411; 72.6%). The median duration of disease at the time of pulmonary fibrosis (PF) detection was 2.5 years, pulmonary arterial hypertension 8.1 years, and renal crisis 4.1 years. By GEE analysis, dcSSc was significantly associated with salt-and-pepper skin, hand deformity, and every 1-point increase in modified Rodnan skin score (mRSS). A greater mortality risk was associated with age at onset >60 years (hazards ratio [HR] 5.5), a World Health Organization functional class (FC) III (HR 5.1), FC IV (HR 34.8), edematous skin (HR 11.4), early onset of PF (HR 1.7), each 5-point increase in the mRSS (HR 4.5), and >= 2 internal organ involvements (HR 10.1).Conclusion dcSSc is a common SSc subset among Thais. PF was an early complication in SSc and earlier PF detection was associated with a poorer prognosis. Elderly onset, high FC, severe skin tightness, and multiple organ involvements were associated with a greater mortality risk.