Juvenile onset recurrent respiratory papillomatosis (JORRP) is a rare but serious disease characterized by recurrent growth of wart-like lesions in the respiratory tract. Papillomas are presumably caused by vertical transmission of human papillomavirus (HPV) types 6 or 11, types which also cause genital warts. In the United States, both quadrivalent and 9-valent HPV vaccine, introduced in 2006 and 2015, respectively, protect against infection with these HPV types 6 and 11. HPV vaccination is routinely recommended for U.S. adolescents at age 11 or 12 years, with catch-up vaccination recommended for females through age 26 years. We aimed to establish a national registry for monitoring JORRP burden of disease in the HPV vaccine era.
Since January 2015, a prospective, multicenter study has been enrolling patients aged <18 years with JORRP presenting for care at participating U.S. pediatric otolaryngology clinics. Clinical disease history is abstracted from medical records, patient demographics and maternal characteristics are reported by mothers, and tissue and brush biopsy from papilloma specimens are tested for 37 types of HPV DNA using Linear Array. We calculated descriptive statistics including proportions, medians, and interquartile ranges (IQRs) using SAS 9.4.
Through December 2016, 101 prevalent cases of JORRP were reported from 15 participating clinics. Among the 101 case-patients, median age at diagnosis was 3 years (IQR: 2–6 years) and median age at enrollment was 8 years (IQR: 5–12 years). In total, 56 (55.4%) were non-Hispanic white, 20 (19.8%) were non-Hispanic black, and 25 (24.8%) were another or unknown race/ethnicity. About half were male (57, 56.4%). Nearly all (93, 92.1%) had been delivered vaginally, and the majority (64, 63.4%) were first-born children. Among 101 mothers of case-patients, median maternal age at delivery was 21 years (IQR: 19–26 years). Few (10, 9.9%) had a known history of genital warts, and none reported receiving any HPV vaccine before delivery. Among 21 case-patients with available HPV typing results, any HPV was detected in 20 (95.2%); HPV type 6 was detected in 15 (71.4%); and HPV type 11 was detected in 4 (19.0%). None were positive for >1 type of HPV.
JORRP case-patients were commonly first-born children delivered vaginally by unvaccinated young mothers. HPV types 6 or 11 were detected in nearly all case-patients tested, even though most mothers did not report a known history of genital warts. Increasing HPV vaccine uptake in the target age group could prevent or eliminate JORRP caused by vaccine-type HPV infections in the United States. Continued monitoring is ongoing.