Mathai Stephen C, Hummers Laura K, Champion Hunter C, Wigley Fredrick M, Zaiman Ari, Hassoun Paul M, Girgis Reda E
Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Arthritis Rheum. 2009 Feb;60(2):569-77. doi: 10.1002/art.24267.
Pulmonary hypertension (PH) is an important cause of mortality in systemic sclerosis (SSc), where it can be isolated (pulmonary arterial hypertension [PAH]) or associated with interstitial lung disease (ILD). This study was undertaken to characterize determinants of survival among SSc patients with either type of PH who received PAH-specific therapy.
Consecutive SSc patients with PAH or ILD-associated PH confirmed by right heart catheterization were included in the study. Kaplan-Meier and Cox proportional hazards models were used to compare survival between SSc patients with PAH and those with ILD-associated PH and to identify predictors of survival.
Fifty-nine patients (39 with PAH and 20 with ILD-associated PH) were identified. The majority (15 of 20 with ILD-associated PH and 27 of 39 with PAH) received an endothelin receptor antagonist as initial therapy. Median followup time was 4.4 years (range 2.7-7.4 years). Survival was significantly worse in SSc patients with ILD-associated PH than in those with PAH (1-, 2-, and 3-year survival rates 82%, 46%, and 39% versus 87%, 79%, and 64%, respectively; P < 0.01 by log rank test). In a multivariable analysis, ILD-associated PH was associated with a 5-fold increase in risk of death compared with PAH. Pulmonary vascular resistance index was also an independent predictor of mortality in the overall cohort (hazard ratio 1.05, P < 0.01) and was a significant univariable risk factor in each group separately. Type of initial PAH therapy and the use of warfarin were not related to survival.
Survival in SSc complicated by PH remains poor despite currently available treatment options. While therapy may be associated with improved survival in PAH compared with historical controls, the prognosis for patients with ILD-associated PH is particularly grim. Early diagnosis and treatment may improve outcomes since worsening hemodynamic factors were associated with reduced survival.
肺动脉高压（PH）是系统性硬化症（SSc）患者死亡的重要原因，在SSc中，PH可以是孤立性的（肺动脉高压[PAH]），也可以与间质性肺疾病（ILD）相关。本研究旨在确定接受PAH特异性治疗的两种类型PH的SSc患者生存的决定因素。
纳入经右心导管检查确诊为PAH或ILD相关PH的连续性SSc患者。采用Kaplan-Meier法和Cox比例风险模型比较PAH的SSc患者和ILD相关PH的SSc患者的生存率，并确定生存预测因素。
共纳入59例患者（39例PAH患者和20例ILD相关PH患者）。大多数患者（20例ILD相关PH患者中的15例和39例PAH患者中的27例）接受内皮素受体拮抗剂作为初始治疗。中位随访时间为4.4年（范围2.7 - 7.4年）。ILD相关PH的SSc患者的生存率显著低于PAH的SSc患者（1年、2年和3年生存率分别为82%、46%和39%，而PAH患者分别为87%、79%和64%；对数秩检验P < 0.01）。在多变量分析中，与PAH相比，ILD相关PH使死亡风险增加5倍。肺血管阻力指数也是整个队列中死亡率的独立预测因素（风险比1.05，P < 0.01），并且在每组中分别是显著的单变量风险因素。初始PAH治疗类型和华法林的使用与生存无关。
尽管有目前可用的治疗选择，但合并PH的SSc患者的生存率仍然很低。虽然与历史对照相比，治疗可能与PAH患者生存率的改善相关，但ILD相关PH患者的预后尤其严峻。由于血流动力学因素恶化与生存率降低相关，早期诊断和治疗可能改善预后。
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