Mortality and causes of death in a Swedish series of systemic sclerosis patients
Mortality and causes of death in a Swedish series of systemic sclerosis patients
复制标题
DOI：
10.1136/ard.57.11.682
复制
发表时间：
1998-11-01
影响因子：
27.4
通讯作者：
Åkesson, A
中科院分区：
文献类型：
--
作者：
Hesselstrand, R;Scheja, A;Åkesson, A
Objectives-To分析生存率和系统性硬化症（SSc）人群的死亡原因，并评估致命性恶性肿瘤的发生及其与口服环磷酰胺（CYC）treatment.Methods-Survival可能的相关性，计算249例随访13年的SSc患者的生存率。平均（SD）随访时间为5.8（4.2）年。根据死亡原因及其与SSc的关系对49例死亡患者进行了细分。CYC治疗组与非CYC治疗组的恶性肿瘤发生率进行了比较。结果5年和10年生存率分别为86%和69%。与一般人群相比，死亡风险增加了4.6倍。弥漫性皮肤受累（dSSc）和男性亚组的预后比局限性皮肤受累（lSSc）和女性亚组差。在49例死亡中，24例可归因于肺部并发症，如肺纤维化、肺动脉高压、肺炎或肺部恶性肿瘤。口服CYC治疗并没有增加死亡的cancer.Conclusions-Mortality的风险增加，无论是在SSc人口作为一个整体，并在其不同的子集（dSSc和lSSc）。男性dSSc患者的预后最差。然而，5年生存率优于早期研究报告。大多数患者死于心肺疾病。7例致命肺癌中有5例是腺癌，可能是由肺部慢性炎性疾病引起的。在这项研究中，CYC治疗与致死性恶性肿瘤的发生率增加无关。
Objectives-To analyse survival rates and the causes of death in a systemic sclerosis (SSc) population, and to evaluate the occurrence of fatal malignant neoplasms and their possible association with oral cyclophosphamide (CYC) treatment.Methods-Survival was calculated for 249 SSc patients followed up for up to 13 years. Mean (SD) follow up was 5.8 (4.2) years. The 49 deceased patients were subdivided according to causes of death and its relation to SSc. Fatal malignancies in CYC treated patients were compared with those occurring in non-CYC treated patients.Results-The overall 5 and 10 year survival rates were 86% and 69% respectively. There was a 4.6-fold increased risk of death, as compared with the general population. Prognosis was worse in the diffuse cutaneous involvement (dSSc) and male subgroups than in the limited cutaneous involvement (lSSc) and female subgroups. Of the 49 deaths, 24 were attributable to pulmonary complications such as pulmonary fibrosis, pulmonary hypertension, pneumonia or pulmonary malignancy. Treatment with oral CYC did not increase the risk of dying of cancer.Conclusions-Mortality is increased both in the SSc population as a whole and in its different subsets (dSSc and lSSc). Prognosis is worst among male patients with dSSc. However, the 5 year survival rate was better than those reported from earlier studies. Most patients die of cardiopulmonary disease. Five of seven fatal lung cancers were adenocarcinomas, possibly caused by chronic inflammatory disease of the lung. In this study, CYC treatment was not associated with an increased incidence of fatal malignant neoplasms.