Abstract:
Objectives: Juvenile-onset recurrent respiratory papillomatosis (JoRRP) is a rare yet aggressive disease
caused by human papillomavirus (HPV). Although many newborns are likely exposed to HPV, few
develop JoRRP and the clinical course of the disease varies from one child to another. This systematic
review seeks to provide an up-to-date understanding of the risk factors for acquisition and severity.
Methods: We conducted a comprehensive literature search in EMBASE, MEDLINE and EBMR databases
using various combinations of keywords related to JoRRP etiology, risk factors and severity. We also
searched Google Scholar and the reference lists of eligible studies. Our search was limited to original
studies published in French or English between 1995 and July 2012 and to patients under 20 years of age.
Results: Of 1362 citations, we retrieved 102 articles and found 14 additional studies. We retained 32
studies meeting inclusion criteria. All were observational and together included 2296 JoRRP cases. Risk
factors could be classified mainly as maternal and birth history, viral genotype, and host factors. A history
of genital warts during pregnancy and delivery was strongly linked to the development of JoRRP.
Depending on ethnicity, specific human leukocyte antigen class II alleles and immune response factors
were important determinants of JoRRP acquisition and severity. HPV-11 genotype and younger age at
onset of JoRRP were important predictors of severity.
Conclusions: Genetic and immunological profiles underlying the acquisition and clinical course are not
readily modifiable. Thus, preventing condylomas in women of childbearing age could reduce the burden
of this life-threatening disease.
