Hereditary Hemorrhagic Telangiectasia and Aspirin-Exacerbated Respiratory Disease: A Case Report

摘要

While aspirin-exacerbated respiratory disease (AERD) is a routinely encountered diagnosis in the otolaryngology clinic, no previous cases of concomitant AERD and hereditary hemorrhagic telangiectasia (HHT) have been reported. HHT, or Osler-Weber-Rendu disease, is an autosomal dominant disease presenting with mucosal telangiectasias and arteriovenous malformations (AVMs) occurring in an estimated 1 in 2351 to 39,216.1 The role of otolaryngology in the management of these patients routinely involves the treatment of recurrent severe epistaxis. Despite no standard first-line treatment, options include conservative measures, coblation or laser ablation of telangiectasias, and intralesional bevacizumab,1,2 among others. AERD, the triad of aspirin sensitivity, asthma, and nasal polyposis, is another common pathology seen in otolaryngology clinics, occurring in approximately 7% of patients with asthma. Traditional management of these patients with nasal polyps includes endoscopic sinus surgery (ESS) followed by aspirin desensitization.3 However, required daily aspirin therapy and frequent polyp recurrence have led to an appealing medical alternative: dupilumab. While dupilumab has not been specifically studied for AERD, it has been shown to be effective in subgroups of patients with AERD and chronic sinusitis with nasal polyps (CRSwNP).4 The Institutional Review Board of the University of Arkansas for Medical Sciences approved this study (261035).