Abstract
BackgroundRecurrent respiratory papillomatosis (RRP) is a rare respiratory disease primarily caused by chronic human papillomavirus (HPV) infection of serotypes 6 and 11. It manifests in childhood (juvenile-onset recurrent respiratory papillomatosis [JoRRP]) and adulthood (adult-onset recurrent respiratory papillomatosis [AoRRP]), leading to progressive obstruction by papillomas in the upper airway and occasionally in the lower respiratory tract (LRT), including the lungs, with a potential for malignant transformation. This study aimed to delineate the characteristics of JoRRP and AoRRP with LRT involvement in adulthood.MethodsA multicenter French-speaking cohort study was conducted, coupled with a comprehensive literature review of clinical, histological, therapeutic, and prognostic features associated with RRP-LRT.ResultsAmong the 122 with LRT involvement with LRT involvement analyzed, 55 (45%) had JoRRP and 67 (55%) had AoRRP. The mean age at diagnosis was 4 years for JoRRP and 54 years for AoRRP. Ear, nose, and throat involvement was observed in all JoRRP cases and in 34 AoRRP cases (51%). Lung involvement occurred in 47 JoRRP cases (85%) and in ten AoRRP cases (15%). Malignant transformation to squamous cell carcinoma in the trachea (n=6) or lung (n=36) was observed in 42 patients (34%). Factors associated with lung involvement included JoRRP, repeated debulking, and malignant transformation; the only factor associated with malignant transformation was lung involvement. Overall mortality was 16%, with JoRRP, lung involvement, and malignant transformation identified as risk factors for death.ConclusionThis study highlights the prevalence of lung involvement and malignant transformation in RRP with LRT and advocates for targeted screening measures and preventive therapeutic strategies.
Published Version
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