Human papillomavirus (HPV) types 6 and 11 can infect the squamous epithelium of the respiratory tract. Up to 8·9% of patients with HPV-associated recurrent respiratory papillomatosis (RRP) have pulmonary involvement. Pulmonary manifestations of HPV infection are associated with considerable morbidity, in part because treatment options and management guidelines are lacking. Patients with pulmonary RRP have a 32-times increased lifetime risk of malignant transformation compared with the overall RRP population. We review the clinical and radiographic presentation, pathological features, and genetics of pulmonary RRP, and we provide management algorithms based on our clinical experience with this complex patient population. In patients with suspected pulmonary involvement, tissue-sparing procedures to address growing lesions might be warranted given the chronicity and multifocality of the disease over a patient's lifetime. However, malignant transformation of pulmonary lesion(s) warrants standard-of-care treatment for primary lung squamous cell carcinoma. Large cohort studies are needed to understand the clinical course of pulmonary RRP and to identify molecular markers of increased risk of malignant transformation in order to develop guidelines for optimal and standardised surveillance and treatment.

人乳头瘤病毒 (HPV) 6 型和 11 型可感染呼吸道鳞状上皮。高达 8·9% 的 HPV 相关复发性呼吸道乳头状瘤病 (RRP) 患者有肺部受累。HPV 感染的肺部表现与相当大的发病率相关，部分原因是缺乏治疗选择和管理指南。与总体 RRP 人群相比，肺 RRP 患者的终生恶变风险增加 32 倍。我们回顾了肺 RRP 的临床和放射学表现、病理特征和遗传学，并根据我们对这一复杂患者群体的临床经验提供了管理算法。对于疑似肺部受累的患者，考虑到该疾病在患者一生中的慢性性和多灶性，可能需要进行组织保留手术来解决不断增长的病变。然而，肺部病变的恶变需要对原发性肺鳞状细胞癌进行标准护理治疗。需要大型队列研究来了解肺 RRP 的临床病程并确定恶性转化风险增加的分子标志物，以便制定最佳和标准化监测和治疗的指南。