Progressive connective tissue disease (CTD)-related lung disease is a challenging condition that requires lung transplantation in some patients. Treatment with rituximab may improve lung function. To evaluate the effect of rituximab in patients with progressive CTD-related lung disease who met criteria for inclusion in waiting list for a lung transplant. Retrospective study of patients with progressive CTD-related lung disease with criteria for lung transplant (FVC < 60% and/or DLCO < 40%) that started treatment with rituximab because of disease progression. Clinical variables, pulmonary function tests and chest computed tomography were used to monitor the effect of rituximab. The cohort included 18 patients; systemic sclerosis (7), rheumatoid arthritis (5), systemic lupus erythematosus (4), Sjögren syndrome (1) and antisynthetase syndrome (1). The radiologic patterns observed were: usual interstitial pneumonia (1), non-specific interstitial pneumonia (9), lymphoid interstitial pneumonia (1), emphysema-usual interstitial pneumonia (1), shrinking lung syndrome (3) and undetermined pattern (3). Over the previous year to rituximab initiation a decline in FVC (− 3.8%, p = 0.095) and DLCO (− 8.4%, p = 0.004) was observed. After 2 years of treatment, DLCO significantly improved (+ 12.4%, p < 0.001 at 1 year and + 15.3%, p = 0.001 at 2 years). Six patients (33.3%) presented adverse events related to rituximab. No patient required lung transplant or died during the study period. Rituximab is an effective treatment for patients with severe and progressive CTD-related lung disease, which allows to delay lung transplantation in some cases.

进行性结缔组织病（CTD）相关的肺部疾病是一项具有挑战性的疾病，某些患者需要进行肺移植。利妥昔单抗治疗可改善肺功能。为了评估利妥昔单抗在进展期CTD相关性肺疾病患者中的疗效，这些患者符合纳入肺移植等待名单的标准。进行性CTD相关性肺疾病患者的回顾性研究，其标准为因疾病进展而开始使用利妥昔单抗治疗的肺移植标准（FVC <60％和/或DLCO <40％）。临床变量，肺功能检查和胸部计算机断层扫描用于监测利妥昔单抗的疗效。该队列包括18例患者；系统性硬化症（7），类风湿性关节炎（5），系统性红斑狼疮（4），Sjögren综合征（1）和抗合成酶综合征（1）。所观察到的放射学特征为：普通间质性肺炎（1），非特异性间质性肺炎（9），淋巴样间质性肺炎（1），肺气肿-通常间质性肺炎（1），肺部综合征（3）和不确定的特征（3） 。与利妥昔单抗启动前一年相比，FVC下降了（-3.8％，p  ＝ 0.095）和DLCO（-8.4％，p  ＝ 0.004）。治疗2年后，DLCO显着改善（ 1年时+ 12.4％，p <0.001； 2年时+ 15.3％，p  = 0.001）。6名患者（33.3％）出现了与利妥昔单抗相关的不良事件。在研究期间，没有患者需要进行肺移植或死亡。利妥昔单抗对患有严重和进行性CTD相关性肺疾病的患者有效，在某些情况下可延迟肺移植。