Fever and Cough in a 33-Year-Old Man With Recurrent Respiratory Papillomatosis

Abstract

INTRODUCTION: Recurrent respiratory papillomatosis (RRP) is a viral disease that typically affects the larynx, however in rare cases can involve pulmonary parenchyma. We report a case of RRP with pulmonary metastasis. CASE PRESENTATION: A 33-year-old man, with past medical history of RRP diagnosed at age 28, requiring serial debridement of papillomas, presented with fever, night sweats and productive cough. Two days prior he underwent bronchoscopy with laser destruction of recurrent papillomas of the nasopharynx, larynx, and trachea. It was complicated by post-procedure nausea and emesis. On admission, he was febrile (104.3F) and tachycardic (130 bpm). SpO2 was 94% on room air. CXR and chest CT revealed multiple solid and cavitary nodules with air-fluid levels. AFB, fungal and auto-immune assays were unrevealing. He was initially treated with vancomycin and piperacillin/tazobactam, but converted to ciprofloxacin based on a sputum culture that grew Serratia. Symptomatically he improved; he will be followed closely in pulmonary clinic with repeat CT scan. DISCUSSION: RRP is the result of proliferation of squamous papillomas in the aerodigestive tract. It is associated with HPV-6 and HPV-11. Two forms of disease are recognized: an aggressive form arising early in life, likely secondary to vertical transmission through an infected birth canal, and a less aggressive form typically arising in adulthood. Of interest, this patient's mother died of cervical cancer at age 48. Pulmonary papillomatosis has mainly been reported in children with aggressive disease or after multiple laryngeo-tracheal interventions. It predisposes to recurrent lower respiratory tract infections (LRTI), nodulo-cystic and cavitary lung lesions and respiratory failure due to parenchymal damage. Transformation to squamous cell carcinoma has been reported in 1-2% of patients treated over 10 years. Surgery is the mainstay of treatment for laryngeal involvement, however adjuvant medical treatment is indicated for rapid regrowth of lesions, airway compromise, or distal involvement. Trial data is limited but various therapies have been studied including interferon alpha, anti-virals, photodynamic therapy, retinoids, COX-2 inhibitors, and tyrosine kinase inhibitors. CONCLUSIONS: LRTI in a patient with RRP should prompt evaluation for broncho-pulmonary progression. Pulmonary papillomatosis is a severe complication of RRP and can be associated with malignant transformation.