Background

Sarcoidosis is a multisystem, chronic, progressive, granulomatous disease. Sarcoidosis-associated pulmonary hypertension is a well described, but not common, complication of sarcoidosis. In small scale studies, it has been previously described as manifestation of advanced disease and was found to be associated increased morbidity and mortality. This study sought to assess the long-term prognostic significance of sarcoidosis-associated pulmonary hypertension (SAPH) by using data obtained from a large population-based registry which contains longitudinal follow-up data.

Methods

Utilizing the records of the largest healthcare provider in Israel, we extracted a cohort consisting of sarcoidosis patients and age-and-sex matched controls. Dates of sarcoidosis registration, pulmonary hypertension and death, as well as anthropometric information and medical comorbidities, were extracted from the database. A multivariate logistic regression model was used to find variables associated with pulmonary hypertension. Cox proportional hazards method and log-rank test were used for survival analysis.

Results

The cohort included 3993 sarcoidosis patients and 19,856 controls. Pulmonary hypertension was observed among 269 sarcoidosis patients (6.74%) vs. 400 controls (2.01%). Sarcoidosis was found as independently associated with pulmonary hypertension (OR 3.17). After a mean follow-up of 7.49 years (median 7.24, maximum 17.88 years), 710 (17.8%) of the sarcoidosis patients and 2121 (10.7%) of the controls had died. Both sarcoidosis and pulmonary hypertension were found to be significantly associated with an increased risk of all-cause mortality (HR 1.82 and HR 2.31, respectively).

Conclusions

SAPH is associated with a poor prognosis. Proper screening methods may assess whether early identification and treatment improve life expectancy.

中文翻译：

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结节病相关性肺动脉高压的长期预后意义–队列研究

背景

结节病是一种多系统，慢性，进行性肉芽肿性疾病。结节病相关的肺动脉高压是结节病的一个很好描述的但不常见的并发症。在小规模研究中，先前已将其描述为晚期疾病的表现，并发现其与发病率和死亡率增加相关。这项研究试图使用结直肠癌相关的肺动脉高压（SAPH）的长期预后意义，该数据使用的是基于大规模人群登记的纵向随访数据。

方法

利用以色列最大的医疗服务提供者的记录，我们提取了一个由结节病患者和年龄和性别匹配的对照组组成的队列。从数据库中提取结节病登记，肺动脉高压和死亡的日期以及人体测量学信息和合并症。使用多元逻辑回归模型来查找与肺动脉高压相关的变量。生存率分析采用Cox比例风险法和对数秩检验。

结果

该队列包括3993例结节病患者和19856例对照。269例结节病患者中观察到肺动脉高压（6.74％），而400例对照组中观察到肺动脉高压（2.01％）。发现结节病与肺动脉高压独立相关（OR 3.17）。在平均随访7.49年（中位数7.24，最大17.88年）后，结节病患者710例（17.8％）和对照组死亡2121例（10.7％）。发现结节病和肺动脉高压均与全因死亡风险增加显着相关（分别为HR 1.82和HR 2.31）。

结论

SAPH与不良预后相关。正确的筛查方法可以评估早期识别和治疗是否可以改善预期寿命。