Service d'Oncologie Thoracique, Hôpital Bichat, 46 rue Henri Huchard, 75018, Paris, France.
CIC INSERM 1425-CLIP2 Paris-Nord, Hôpital Bichat-Claude Bernard, 46 rue Henri Huchard 75018, AP-HP, Paris, France.
BMC Pulm Med. 2019 Jan 11;19(1):12. doi: 10.1186/s12890-018-0775-5.
Sporadic lymphangioleiomyomatosis (LAM) is a rare form of diffuse parenchymal lung disease. PD-1 blocking antibodies constitute an essential treatment option for advanced non-small-cell lung cancer (NSCLC). The effect of immune checkpoint inhibitors in lymphangioleiomyomatosis patients with non-small cell lung cancer is unknown: concomitant symptomatic interstitial lung disease or the use of immunosuppressors was a key exclusion criterion in the original studies of immune checkpoint inhibitors, especially regarding the risk of interstitial lung disease exacerbation.
A 48-year-old female, active smoker (36 pack-years), diagnosed with sporadic LAM since 2004 suffered from metastatic adenocarcinoma of the lung. Third-line therapy with nivolumab was started in 2015, with a major partial response. Due to pulmonary function alterations, sirolimus was also reinitiated in 2017 in conjunction with nivolumab, without any undesirable effects and a major partial response continuing up to May 2018.
This case highlights the safe and effective use of nivolumab for managing metastatic lung adenocarcinoma that occurred in a patient with sporadic LAM. In the current case, immunotherapy proved highly successful in managing the NSCLC tumor that occurred upon LAM follow-up, with both a significantly prolonged partial response and acceptable safety profile.
散发性淋巴管平滑肌瘤病（LAM）是一种罕见的弥漫性实质肺疾病。PD-1 阻断抗体是晚期非小细胞肺癌（NSCLC）的重要治疗选择。免疫检查点抑制剂在伴有非小细胞肺癌的淋巴管平滑肌瘤病患者中的作用尚不清楚：同时存在症状性间质性肺病或使用免疫抑制剂是免疫检查点抑制剂原始研究中的关键排除标准，特别是关于间质性肺病恶化的风险。
一名 48 岁女性，吸烟者（36 包年），自 2004 年以来被诊断为散发性 LAM，患有转移性肺腺癌。2015 年开始接受三线治疗用nivolumab，主要部分缓解。由于肺功能改变，2017 年与 nivolumab 一起重新开始使用西罗莫司，没有任何不良反应，主要部分缓解持续到 2018 年 5 月。
本病例强调了 nivolumab 用于管理散发性 LAM 患者中发生的转移性肺腺癌的安全性和有效性。在目前的情况下，免疫疗法在管理 LAM 随访中发生的 NSCLC 肿瘤方面非常成功，具有显著延长的部分缓解和可接受的安全性。
