Department of Rheumatology (Planta 10-2), Servicio de Reumatología, Hospital Universitario de Bellvitge, Feixa Llarga, s/n, Hospitalet de Llobregat, 08907, Barcelona, Spain.
Unidad de Investigación, Sociedad Española de Reumatología, Madrid, Spain.
Arthritis Res Ther. 2018 Dec 19;20(1):280. doi: 10.1186/s13075-018-1776-8.
The purpose of this study was to assess the prevalence, associated factors, and impact on mortality of primary respiratory disease in a large systemic lupus erythematosus (SLE) retrospective cohort.
All adult patients in the RELESSER-TRANS (Registry of Systemic Lupus Erythematosus Patients of the Spanish Society of Rheumatology [SER], cross-sectional phase) registry were retrospectively investigated for the presence of primary pleuropulmonary manifestations.
In total 3215 patients were included. At least one pleuropulmonary manifestation was present in 31% of patients. The most common manifestation was pleural disease (21%), followed by lupus pneumonitis (3.6%), pulmonary thromboembolism (2.9%), primary pulmonary hypertension (2.4%), diffuse interstitial lung disease (2%), alveolar hemorrhage (0.8%), and shrinking lung syndrome (0.8%). In the multivariable analysis, the variables associated with the development of pleuropulmonary manifestation were older age at disease onset (odds ratio (OR) 1.03, 95% confidence interval (CI) 1.02-1.04), higher SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) scores (OR 1.03, 95% CI 1.00-1.07), the presence of Raynaud's phenomenon (OR 1.41, 95% CI 1.09-1.84), secondary antiphospholipid syndrome (OR 2.20, 95% CI 1.63-2.97), and the previous or concomitant occurrence of severe lupus nephritis, (OR 1.48, 95% CI 1.12-1.95) neuropsychiatric manifestations (OR 1.49, 95% CI 1.11-2.02), non-ischemic cardiac disease (OR 2.91, 95% CI 1.90-4.15), vasculitis (OR 1.81, 95% CI 1.25-2.62), hematological manifestations (OR 1.31, 95% CI 1.00-1.71), and gastrointestinal manifestations, excluding hepatitis (OR 2.05, 95% CI 1.14-3.66). Anti-RNP positivity had a clear tendency to significance (OR 1.32, 95% CI 1.00-1.75; P = 0.054). The development of pleuropulmonary manifestations independently contributes to a diminished survival (hazard ratio of 3.13). However, not all complications will influence the prognosis in the same way. Whereas the occurrence of pleural disease or pulmonary thromboembolism has a minimal impact on the survival of these patients, the remaining manifestations have a major impact on mortality.
Except for pleural disease, the remaining respiratory manifestations are very uncommon in SLE (<4%). Pleuropulmonary manifestations independently contributed to a decreased survival in these patients.
本研究旨在评估大型系统性红斑狼疮（SLE）回顾性队列中主要呼吸系统疾病的患病率、相关因素及对死亡率的影响。
对西班牙风湿病学会（SER）系统性红斑狼疮患者登记处（RELESSER-TRANS [登记处]）的所有成年患者进行回顾性调查，以确定是否存在原发性胸膜肺表现。
共纳入 3215 例患者。31%的患者至少存在一种胸膜肺表现。最常见的表现是胸膜疾病（21%），其次是狼疮性肺炎（3.6%）、肺血栓栓塞症（2.9%）、原发性肺动脉高压（2.4%）、弥漫性间质性肺病（2%）、肺泡出血（0.8%）和缩窄性肺综合征（0.8%）。多变量分析显示，与胸膜肺表现发生相关的变量为发病年龄较大（比值比（OR）1.03，95%置信区间（CI）1.02-1.04）、SLEDAI 评分较高（OR 1.03，95%CI 1.00-1.07）、存在雷诺现象（OR 1.41，95%CI 1.09-1.84）、继发性抗磷脂综合征（OR 2.20，95%CI 1.63-2.97）以及先前或同时发生严重狼疮肾炎（OR 1.48，95%CI 1.12-1.95）、神经精神表现（OR 1.49，95%CI 1.11-2.02）、非缺血性心脏疾病（OR 2.91，95%CI 1.90-4.15）、血管炎（OR 1.81，95%CI 1.25-2.62）、血液学表现（OR 1.31，95%CI 1.00-1.71）和胃肠道表现，不包括肝炎（OR 2.05，95%CI 1.14-3.66）。抗 RNP 阳性有明显的显著趋势（OR 1.32，95%CI 1.00-1.75；P=0.054）。胸膜肺表现的发生独立导致生存时间缩短（风险比为 3.13）。然而，并非所有并发症都会以同样的方式影响预后。虽然胸腔疾病或肺血栓栓塞症的发生对这些患者的生存影响较小，但其余表现对死亡率有重大影响。
除胸腔疾病外，SLE 中其余呼吸系统表现非常少见（<4%）。胸膜肺表现独立导致这些患者的生存时间缩短。
