A Clinical Consensus Statement on Pulmonary Recurrent Respiratory Papillomatosis

Pulmonary recurrent respiratory papillomatosis (pRRP) is a rare manifestation of RRP with a paucity of data on workup and management. The objective was to develop guidance on the screening, diagnosis, and management of pRRP based on literature review and multidisciplinary expert consensus.

A pRRP expert group was assembled, comprising one representative from the RRP Foundation and 19 adult and pediatric otolaryngologists, thoracic surgeons, pulmonologists, oncologists, pathologists, and radiologists with experience in the management of pRRP. A rigorous Delphi method including video meetings and consensus surveys was used to establish consensus pertaining to screening, diagnosis, management, and treatment.

After the literature review, the core working group created 33 statements regarding screening, diagnosis, management, and treatment. After distribution to the consensus group, three iterative surveys were performed, each followed by 90 min of live discussion. Consensus was achieved for screening (9 statements); diagnosis (6); management (4); treatment (11); and systemic treatment (3). Key statements include a recommendation for HPV typing at the time of diagnosis; chest CT to monitor disease progression; biopsy of suspected pulmonary lesions when possible; a multidisciplinary team for management; and consideration of systemic therapy.

pRRP is present in up to 9% of patients with RRP and carries a 32-fold increased risk of malignant transformation. Obtaining consensus on best practices can reduce variability in the treatment of orphan diseases and guide future research efforts. This statement reflects the efforts of a national group of dedicated experts with an established history in managing this difficult disease.

Level V.