Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
CKDnet, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
Int J Rheum Dis. 2020 Jul;23(7):945-957. doi: 10.1111/1756-185X.13859. Epub 2020 May 18.
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.
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.
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).
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).
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.
与大多数白人和一些亚洲人不同，大多数泰国系统性硬化症（SSc）患者为弥漫性皮肤型 SSc（dcSSc）。需要对泰国 dcSSc 患者进行纵向队列研究。
我们旨在确定总体临床特征，定义局限性皮肤型 SSc（lcSSc）和 dcSSc 之间的临床差异，并确定死亡率和与死亡率相关的因素。
我们进行了一项队列研究，纳入了 2013 年 1 月至 2019 年 6 月期间的 566 例泰国成年 SSc 患者。使用广义估计方程（GEE）来研究 lcSSc 和 dcSSc 之间的临床差异。
女性多于男性（356 例比 210 例）。大多数患者为 dcSSc（411 例，占 72.6%）。在检测到肺纤维化（PF）时疾病的中位病程为 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 与椒盐皮肤、手部畸形和改良 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）、水肿性皮肤（HR 11.4）、PF 早期发病（HR 1.7）、mRSS 每增加 5 分（HR 4.5）和≥2 个内脏器官受累（HR 10.1）与更高的死亡风险相关。
dcSSc 是泰国 SSc 的常见亚型。PF 是 SSc 的早期并发症，更早的 PF 检测与更差的预后相关。发病年龄较大、FC 较高、严重的皮肤紧绷和多个器官受累与更高的死亡风险相关。
