Successful treatment of juvenile laryngeal papillomatosis-related multicystic lung disease with cidofovir - Case report and review of the literature

Cidofovir, a nucleoside analog antiviral agent, has been used with moderate success in the treatment of juvenile laryngeal papillomatosis (JLP) by dir ect intralesional injection. We report the first case where IV cidofovir wa s used successfully to treat a rare but lethal multicystic lung disease com plicating JLP. A 35-year-old woman with a history of JLP requiring multiple laser ablations of laryngeal papillomata each year presented with hemoptys is and was found on CT scan to have bilateral, multiple pulmonary nodules a nd cysts. The results of BAL fluid analysis demonstrated no evidence of mal ignancy, and cultures were negative for fungi and mycobacteria. Molecular D NA typing of a biopsy specimen obtained from a laryngeal papilloma confirme d infection with human papilloma virus type 11. She received 12 months of t reatment with IV cidofovir followed by 9 months of combined treatment with IV cidofovir and subcutaneous interferon-alpha -2A. This therapeutic regime resulted in a markedly decreased requirement for surgical removal of laryn geal papillomata, and CT scanning documented the regression of the lesions in the lung parenchyma that persisted after the discontinuation of therapy. The results of this case demonstrate that cidofovir may be used successful ly to treat JLP-related lung disease and suggest that further studies are w arranted.