Department of Pulmonology, Semmelweis University, Budapest, Hungary.
Medical Imaging Centre, Semmelweis University, Budapest, Hungary.
Thorac Cancer. 2020 Jul;11(7):1911-1917. doi: 10.1111/1759-7714.13481. Epub 2020 May 13.
Fibrosing interstitial lung diseases (ILDs) are associated with poor survival and an increased risk of developing lung cancer (LC). Patient and LC characteristics, therapeutic possibilities and survival in this rare patient population are not well established.
Fibrosing ILD patients treated at the Department of Pulmonology Semmelweis University were reviewed retrospectively between 2012-2018 (N = 160). All patients with concomitant LC (N = 23) underwent detailed pulmonary evaluation. Cancer characteristics including driver mutation data, as well as therapy and survival were analyzed.
ILD-LC patients (56% men, mean age 73 ± 6 years) had mild-moderate lung functional impairment (forced vital capacity [FVC]: 80 ± 24%ref., forced expiratory volume in one second [FEV1]: 76 ± 27%ref.; transfer factor of the lung for carbon monoxide [TLCO]: 62 ± 25% reference). In 56% of cases histology confirmed adenocarcinoma followed by squamous cell carcinoma in 26%. Lobectomy could only be performed in one case; driver mutation was present in one patient. Chemotherapy was most commonly administered; however, 26% could only receive supportive palliative care. Four idiopathic pulmonary fibrosis patients received concomitant nintedanib to their LC treatment. Median survival of ILD-LC patients was only 321 days.
Diagnosis and therapy of ILD-LC is challenging and patients have a very limited survival. A significant proportion of patients could only receive palliative care indicating the need for better management strategies in this special patient population. The evaluation of the effect of cotreatment with antifibrotics needs further study.
Interstitial lung diseases are often associated with lung cancer Diagnosis is challenging and therapy often limited due to underlying lung disease. Patients received platinum based chemotherapy or only supportive care.
纤维化间质性肺疾病（ILDs）与生存不良和肺癌（LC）风险增加相关。该罕见患者人群的患者和 LC 特征、治疗可能性和生存情况尚不清楚。
对 2012-2018 年在森梅威思大学肺病科接受治疗的纤维化 ILD 患者进行回顾性分析（N=160）。所有合并 LC（N=23）的患者均进行了详细的肺部评估。分析了癌症特征，包括驱动基因突变数据，以及治疗和生存情况。
ILD-LC 患者（56%为男性，平均年龄 73±6 岁）存在轻中度肺功能障碍（用力肺活量 [FVC]：80±24%参考值，第一秒用力呼气量 [FEV1]：76±27%参考值；一氧化碳肺转移系数 [TLCO]：62±25%参考值）。组织学证实 56%为腺癌，其次为鳞状细胞癌（26%）。仅有 1 例患者行肺叶切除术；1 例患者存在驱动基因突变。最常采用的治疗方法为化疗，但 26%的患者仅能接受姑息支持治疗。4 例特发性肺纤维化患者在接受 LC 治疗的同时还接受了尼达尼布治疗。ILD-LC 患者的中位生存时间仅为 321 天。
ILD-LC 的诊断和治疗具有挑战性，患者的生存时间非常有限。由于基础肺病的存在，很大一部分患者只能接受姑息治疗，这表明该特殊患者群体需要更好的管理策略。评估抗纤维化药物联合治疗的效果需要进一步研究。
间质性肺疾病常与肺癌相关诊断具有挑战性，且由于基础肺病的存在，治疗往往受限。患者接受铂类为基础的化疗或仅接受支持性治疗。
