Rubio-Rivas Manuel, Simeón-Aznar Carmen Pilar, Velasco César, Marí-Alfonso Begoña, Espinosa Gerard, Corbella Xavier, Colunga-Argüelles Dolores, Egurbide-Arberas Maria Victoria, Ortego-Centeno Norberto, Vargas-Hitos José Antonio, Freire Mayka, Ríos-Blanco Juan José, Trapiella-Martínez Luis, Rodríguez-Carballeira Mónica, Fonollosa-Pla Vicent
Autoimmune Diseases Unit, Department of Internal Medicine, Bellvitge University Hospital-IDIBELL, L'Hospitalet de Llobregat, Barcelona, Spain.
Vall d'Hebron University Hospital, Department of Internal Medicine, Barcelona, Spain.
Clin Exp Rheumatol. 2017 Sep-Oct;35 Suppl 106(4):40-47. Epub 2017 Feb 6.
To determine the changes in the pattern of death of patients with systemic sclerosis (SSc) throughout 20 years.
Data were collected from the Spanish Scleroderma Registry (RESCLE), retrospective multicentre database from 1990 to 2009. SSc-related and SSc-non related causes of death were assessed.
987 patients were recruited. Overall standardised mortality ratio (SMR) was 2.34 (2.24-2.44). SSc-related causes of death were responsible of 72% of all deaths of those patients diagnosed within 1990-99 vs. 48% within 2000-09 (p=0.006). Relative pulmonary death rate was stable over time (68.1% within 1990-99 vs. 63.9% within 2000-09, p=0.815). Relative renal death rate was decreasing over time (17% within 1990-99 vs. 5.5% within 2000-09, p=0.175). Heart distribution tripled its ratio (12.8% within 1990-99 vs. 30.6% within 2000-09, p=0.058).
SSc-related causes of death were decreasing over time and, among them, pulmonary involvement was the leading cause of death in both decades. The ratio of renal causes decreased since 1990 at the time that the ratio of cardiac causes increased.
确定20年间系统性硬化症（SSc）患者的死亡模式变化。
数据收集自西班牙硬皮病注册中心（RESCLE），这是一个1990年至2009年的回顾性多中心数据库。评估了与SSc相关和与SSc无关的死亡原因。
招募了987名患者。总体标准化死亡率（SMR）为2.34（2.24 - 2.44）。1990 - 1999年诊断的患者中，与SSc相关的死亡原因占所有死亡的72%，而2000 - 2009年为48%（p = 0.006）。相对肺部死亡率随时间保持稳定（1990 - 1999年为68.1%，2000 - 2009年为63.9%，p = 0.815）。相对肾脏死亡率随时间下降（1990 - 1999年为17%，2000 - 2009年为5.5%，p = 0.175）。心脏相关死亡比例增加了两倍（1990 - 1999年为12.8%，2000 - 2009年为30.6%，p = 0.058）。
与SSc相关的死亡原因随时间减少，其中肺部受累是这两个十年中的主要死亡原因。自1990年以来，肾脏相关原因的比例下降，而心脏相关原因的比例增加。
