Recurrent respiratory papillomatosis (RRP) is a rare disease. In children vertical transmission of HPV is thought to be the causative agent. Although HPV has also been found in adults its route of transmission is less clear. The role of immune deficiency as a risk factor for disease development has not yet been determined. We will be presenting an adult patient with acquired immunodeficiency syndrome (AIDS) with RRP and distal dissemination.
A 64 year old male with AIDS (CD4 116) who presented with recurrent episodes of hemoptysis over a three month period. He also reported a productive cough, night sweats, and 15 lb weight loss. He had been ruled out for tuberculosis and treated for pneumonia during a recent admission for similar symptoms. On physical exam he was afebrile but had bilateral basilar rhonchi. Routine laboratory tests were unremarkable. Chest x-ray revealed patchy consolidation of the right mid and lower lobes with CT chest confirming these areas of consolidation as well as revealing a large endotracheal lesion. PET CT showed increased uptake in the trachea and areas of consolidation. Subsequent fiberoptic and virtual bronchoscopies demonstrated numerous exophytic polyps lining the entire trachea down to the carina. Histology was positive for squamous papilloma fragments and the presence of low risk strains of HPV was confirmed by HPV-DNA testing. Upon further review we found that he had first been diagnosed with tracheal squamous papillomas two years prior but unfortunately was lost to follow-up while caring for his wife who had been diagnosed with cervical cancer. During this admission there had been significant progression of the papillomas and distal dissemination and malignant transformation were concerns. CT-guided RLL lung biopsy revealed dysplastic squamous epithelium with foci of squamous metaplasia confirming our diagnosis of RRP with distal parenchymal dissemination.
RRP is a rare disease. Although more commonly diagnosed in children with airway obstruction there have been reported adult onset cases. In children vertical transmission of HPV is thought to be the causative agent. Although in adults HPV has also been found in these lesions the route of transmission is less clear. Oro-genital sexual transmission seems like the most likely culprit with low risk HPV strains 6 and 11. Treatment involves surgical laser resection. Other options include interferon-alpha, antivirals, cimetidine, and celebrex all of which are being studied. Even with surgical treatment lesions tend to recur. Most will require multiple surgical interventions to control disease complications. Distal spread has been reported into the smaller bronchial airways and lung parenchyma. Malignant transformation into squamous cell carcinoma is also a major concern. PET CT does not seem to be useful for accessing malignant transformation since increased uptake is also present in the paillomas. Currently no recommended screening guidelines exist. Another area of uncertainty is whether AIDS itself is a risk factor for RRP. There has been a case report of disease development after immune reconstitution with highly active antiretroviral therapy (HAART). However our patient had low CD4 counts and known noncompliance with HAART. We propose that this patient’s immune deficiency increased his risk for development of RRP with distal dissemination.
1. RRP is caused by low risk HPV stains.2. RRP may present with endotracheal lesions and distal dissemination may present as pneumonic infiltrates. 3. PET scan is not useful in accessing malignant transformation. 4. In adult onset RRP AIDS may be a risk factor.
Ami Abraham, No Financial Disclosure Information; No Product/Research Disclosure Information