Department of Rheumatology, Loyola University Medical Center, Louisville, KY 40207, USA.
J Clin Rheumatol. 2012 Sep;18(6):283-9. doi: 10.1097/RHU.0b013e3182676089.
Connective tissue disease (CTD)-related lung dysfunction is a common cause of morbidity and mortality; however, few lung transplantations (LTs) are performed in this population secondary to uncertainty regarding the posttransplant survival, outcome, and management.
The objectives were to evaluate the survival and the pulmonary and extrapulmonary courses of CTD after LT.
Survival outcomes of patients documented within the Organ Procurement and Transplantation Network who had undergone a LT for CTD were compared with those who underwent LT for chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). In addition, the pulmonary and extrapulmonary courses of the CTD were evaluated after LT.
From 1991 to 2009, there were 284 documented LT in patients with CTD. Post-LT cumulative survival of patients with CTD was less than that for COPD through 5 years, with a difference that peaked at 1 year (72.7% vs. 83.1%, P < 0.001). When patients with CTD were compared with those with IPF, a difference was only noted at 1 year (72.7% vs. 77.7%, P = 0.049). There were no documented post-LT pulmonary recurrences of the CTD, and extrapulmonary flares of the CTD were rare (1 possible flare per 20.3 patient-years and 1 probable flare per 81.0 patient-years).
Cumulative survival of patients with CTD who underwent LT is similar to those with IPF and slightly less than those with COPD, with an increased risk of mortality that was most prominent at 6 months after transplant followed by subsequent narrowing of the survival differences over time. Lung transplantation may be a viable therapeutic option for patients with end-stage lung dysfunction resulting from a CTD.
结缔组织病（CTD）相关的肺功能障碍是发病率和死亡率的常见原因；然而，由于对移植后生存、结果和管理的不确定性，该人群中很少进行肺移植（LT）。
评估 LT 后 CTD 的生存和肺外表现。
比较了在器官获取和移植网络中记录的接受 LT 治疗 CTD 的患者的生存结果与接受 LT 治疗慢性阻塞性肺疾病（COPD）和特发性肺纤维化（IPF）的患者。此外，还评估了 LT 后 CTD 的肺外表现。
1991 年至 2009 年，有 284 例 CTD 患者接受了 LT。LT 后 CTD 患者的累积生存率低于 COPD 患者，5 年内差异最大，1 年时达到峰值（72.7%比 83.1%，P <0.001）。当 CTD 患者与 IPF 患者进行比较时，仅在 1 年时观察到差异（72.7%比 77.7%，P = 0.049）。未发现 LT 后 CTD 的肺复发，且 CTD 的肺外发作罕见（每 20.3 例患者年有 1 例可能的发作，每 81.0 例患者年有 1 例可能的发作）。
LT 治疗的 CTD 患者的累积生存率与 IPF 相似，略低于 COPD，移植后 6 个月时死亡率风险增加，随后随着时间的推移，生存差异逐渐缩小。LT 可能是终末期 CTD 导致的肺功能障碍患者的一种可行的治疗选择。
