Schachna Lionel, Medsger Thomas A, Dauber James H, Wigley Fredrick M, Braunstein Neil A, White Barbara, Steen Virginia D, Conte John V, Yang Stephen C, McCurry Kenneth R, Borja Marvin C, Plaskon David E, Orens Jonathan B, Gelber Allan C
Johns Hopkins University, Baltimore, Maryland 21224, USA.
Arthritis Rheum. 2006 Dec;54(12):3954-61. doi: 10.1002/art.22264.
Lung transplantation is a viable, life-saving intervention for several primary pulmonary disorders complicated by severe lung dysfunction. This study was undertaken to evaluate whether patients with systemic sclerosis (scleroderma), a systemic autoimmune rheumatic disorder, would receive similar benefit from this intervention.
Survival following lung transplantation was examined at 2 university medical centers among 29 patients with scleroderma as compared with 70 patients with idiopathic pulmonary fibrosis (IPF) and 38 with idiopathic pulmonary arterial hypertension (IPAH), the latter groups representing pathologically related primary pulmonary disorders. The end point was death from any cause. Risk of mortality in patients with scleroderma was compared with that in patients with IPF or IPAH, with adjustment for demographic and clinical parameters.
During 2 years of followup, 11 patients with scleroderma (38%), 23 with IPF (33%), and 14 with IPAH (37%) died. Cumulative survival at 6 months posttransplantation was 69% in the scleroderma group compared with 80% in the IPF group (log-rank P = 0.21) and 79% in the IPAH group (P = 0.38). The estimated risk of mortality at 6 months was increased in patients with scleroderma compared with those with IPF (relative risk [RR] 1.70, 95% confidence interval [95% CI] 0.74-3.93) and those with IPAH (RR 1.52, 95% CI 0.59-3.96), but the differences were not statistically significant. Over the following 18 months, there was convergence in the survival rates such that cumulative survival at 2 years was comparable, at approximately 64%, among all 3 groups.
Patients with scleroderma who are recipients of lung transplantation experience similar rates of survival 2 years after the procedure compared with those with IPF or IPAH. Lung transplantation may represent a viable therapeutic option to consider for patients with end-stage lung disease due to scleroderma.
肺移植是对几种并发严重肺功能障碍的原发性肺部疾病可行的挽救生命的干预措施。本研究旨在评估系统性硬化症（硬皮病）患者（一种系统性自身免疫性风湿性疾病）是否能从该干预措施中获得类似益处。
在2所大学医学中心对29例硬皮病患者的肺移植术后生存率进行了研究，并与70例特发性肺纤维化（IPF）患者和38例特发性肺动脉高压（IPAH）患者进行比较，后两组代表病理相关的原发性肺部疾病。终点为任何原因导致的死亡。对硬皮病患者的死亡风险与IPF或IPAH患者的死亡风险进行比较，并对人口统计学和临床参数进行调整。
在2年的随访期间，11例硬皮病患者（38%）、23例IPF患者（33%）和14例IPAH患者（37%）死亡。移植后6个月硬皮病组的累积生存率为69%，而IPF组为80%（对数秩检验P = 0.21），IPAH组为79%（P = 0.38）。与IPF患者（相对风险[RR] 1.70，95%置信区间[95% CI] 0.74 - 3.93）和IPAH患者（RR 1.52，95% CI 0.59 - 3.96）相比，硬皮病患者在6个月时的估计死亡风险增加，但差异无统计学意义。在接下来的18个月中，生存率出现趋同，以至于所有3组在2年时的累积生存率相当，约为64%。
与IPF或IPAH患者相比，接受肺移植的硬皮病患者在术后2年的生存率相似。肺移植可能是因硬皮病导致终末期肺病患者可考虑的一种可行治疗选择。
