Narváez Javier, Aguilar-Coll Martí, Vicens-Zygmunt Vanesa, Alegre Juan José, Bermudo Guadalupe, Molina-Molina María
Department of Rheumatology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain.
Interstitial Lung Disease Unit, Department of Pneumology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain.
J Clin Med. 2024 Nov 22;13(23):7074. doi: 10.3390/jcm13237074.
: Interstitial lung disease (ILD) is one of the most severe complications of rheumatoid arthritis (RA). Real-world data on antifibrotic treatment are needed. Our objective was to evaluate the real-world effectiveness and tolerability of antifibrotic agents in patients with progressive fibrosing RA-ILD. A longitudinal, retrospective, observational study was conducted on a cohort of RA-ILD patients treated with either nintedanib or pirfenidone. The data collected included pulmonary function test (PFT) results, adverse events (AEs), tolerability, and drug retention. Twenty-seven patients were included; 25 (92.5%) initiated nintedanib, while two initiated pirfenidone. The median follow-up duration was 25 months (IQR 7-27). The mean decline in %pFVC and %pDLCO from ILD diagnosis to the initiation of antifibrotic therapy were -8.9% and -14.8%, respectively. After 6 months of treatment, most patients achieved stabilization in PFT: a ∆%pFVC of +1.2% ( = 0.611 compared with baseline) and a ∆%pDLCO of +3.9% ( = 0.400). Eighteen patients completed one year of therapy, with a modest improvement in %pFVC (+4.7%; = 0.023) and stabilization in %pDLCO (-3.8%; = 0.175). This trend persisted among the nine patients who completed 2 years of treatment (%pFVC +7.7%; = 0.037 and %pDLCO -2.2%; = 0.621). During the follow-up period, 15% of patients died, and 4% underwent lung transplantation. Adverse events occurred in 81% of patients, leading to discontinuation in 18.5% of cases. The most frequent adverse events were gastrointestinal events and hepatitis, leading to a permanent dose reduction of 40% for nintedanib and 14% for pirfenidone. A second antifibrotic agent was prescribed for 18.5% of the patients. At the end of the follow-up period, 63% of the total cohort remained on antifibrotic therapy. According to our results, antifibrotic initiation was associated with a modest improvement in the trajectory of %pFVC and stabilization in %pDLCO. The discontinuation rate in our cohort (37%) was higher than that reported in clinical trials but similar to that reported in previously published real-world studies.
间质性肺疾病（ILD）是类风湿关节炎（RA）最严重的并发症之一。需要有关抗纤维化治疗的真实世界数据。我们的目的是评估抗纤维化药物在进行性纤维化RA-ILD患者中的真实世界有效性和耐受性。对一组接受尼达尼布或吡非尼酮治疗的RA-ILD患者进行了一项纵向、回顾性观察研究。收集的数据包括肺功能测试（PFT）结果、不良事件（AE）、耐受性和药物保留情况。纳入了27例患者；25例（92.5%）开始使用尼达尼布，2例开始使用吡非尼酮。中位随访时间为25个月（四分位间距7-27）。从ILD诊断到开始抗纤维化治疗，%pFVC和%pDLCO的平均下降分别为-8.9%和-14.8%。治疗6个月后，大多数患者的PFT达到稳定：%pFVC变化为+1.2%（与基线相比P=0.611），%pDLCO变化为+3.9%（P=0.400）。18例患者完成了一年的治疗，%pFVC有适度改善（+4.7%；P=0.023），%pDLCO保持稳定（-3.8%；P=0.175）。在完成2年治疗的9例患者中，这一趋势持续存在（%pFVC+7.7%；P=0.037，%pDLCO-2.2%；P=0.621）。在随访期间，15%的患者死亡，4%的患者接受了肺移植。81%的患者发生了不良事件，18.5%的病例因此停药。最常见的不良事件是胃肠道事件和肝炎，导致尼达尼布永久剂量减少40%，吡非尼酮永久剂量减少14%。18.5%的患者换用了第二种抗纤维化药物。在随访期结束时，63%的总队列患者仍在接受抗纤维化治疗。根据我们的结果，开始抗纤维化治疗与%pFVC轨迹的适度改善和%pDLCO的稳定有关。我们队列中的停药率（37%）高于临床试验报告的停药率，但与先前发表的真实世界研究报告的停药率相似。
