系統性紅斑狼瘡相關的肺動脈高壓：生存風險研究

研究顯示在肺動脈高壓診斷後，合併肺動脈高壓的系統性紅斑狼瘡患者的5年存活率為83.9%。

研究背景

肺動脈高壓（PAH）是系統性紅斑狼瘡（SLE）少見的一種併發症。

研究方法

在法國肺高壓中心查找所有的SLE及SLE-PAH患者。其中PAH均經右心導管術證實。由SLE專家選擇不伴有PAH的SLE患者101例作為對照組。K-M曲線用來比較兩組之間的生存率。Cox比例風險模型來評估死亡相關預測因素的危險比。

研究結果

登記在冊的SLA-PAH患者69例，本研究包括51例。與對照組相比，兩組在年齡、性別、SLE病程等之間無統計學差異，但SLA-PAH組抗SSA、抗SSB抗體的陽性率較高。PAH診斷通常在SLE診斷後4.9年（範圍2.8-12.9年）。SLE-PAH患者3年和5年存活率分別為89.4%（95%可信區間，76.2%-96.5%）和83.9%（95%可信區間，68.8%-92.1%）。而抗U1RNP抗體陽性的SLE-PAH患者存活率較高（P=0.04）。

研究結論

在PAH診斷後，SLE-PAH患者的5年存活率為83.9%。抗SSA/SSB抗體對於SLA-PAH患者生存是一個危險因素，而抗U1RNP抗體對於其生存率來說是一個保護性因素。

原 文

Abstract

Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus: Results From the French Pulmonary Hypertension Registry.

BACKGROUND:

Pulmonary arterial hypertension (PAH) is a rare complication of systemic lupus erythematosus (SLE).

METHODS:

We identified all patients with SLE and PAH (SLE-PAH) who were enrolled in the French Pulmonary Hypertension Registry with a diagnosis confirmed by right heart catheterization (RHC). A control group of 101 patients with SLE without known PAH was selected from SLE expert centers participating in the Pulmonary Hypertension Registry. Survival was estimated by the Kaplan-Meier method. Hazard ratios associated with potential predictors of death were estimated using Cox proportional hazard models.

RESULTS:

Of the 69 patients with SLE-PAH identified in the French Pulmonary Hypertension Registry, 51 were included in the study. They did not differ from the control group regarding age, sex, or duration of SLE at the time of the analysis but had a higher frequency of anti-SSA and anti-SSB antibodies. The delay between SLE diagnosis and PAH diagnosis was 4.9 years (range, 2.8-12.9) years. The 3- and 5-year overall survival rates were89.4% (95% CI, 76.2%-96.5%) and 83.9% (95% CI, 68.8%-92.1%), respectively. The survival rate was significantly better in patients with anti-U1-RNP antibodies (P = .04).

CONCLUSIONS:

Patients with SLE-PAH have an overall 5-year survival rate of 83.9% after the PAH diagnosis. Anti-SSA/SSB antibodies may be a risk factor for PAH, and the presence of anti-U1-RNP antibodies appears to be a protective factor regarding survival.

出處：Chest. 2018 Jan;153(1):143-151. doi: 10.1016/j.chest.2017.08.014. Epub 2017 Aug 26.

譯者：李治琴 西京醫院臨床免疫科

來源：西京風濕免疫

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