LATE-ONSET CYSTIC FIBROSIS IN AN ELDERLY MALE

Abstract

Introduction Cystic fibrosis (CF) is described as a disease that manifests primarily in childhood, with only 7 percent diagnosed during adulthood. We describe a man with history of infertility and recurrent bronchitis who was diagnosed with CF at age 69. Case Description A 69-year-old with history of recurrent bronchitis since mid-age (once a year, quickly resolved with short course oral antibiotics) presented with refractory cough with yellowish sputum and sinusitis symptoms after a viral upper respiratory infection 18 months prior. He underwent several rounds of antibiotics and steroid taper but his symptoms quickly relapse after completing each treatment course. CT chest showed cylindrical type bronchiectasis. CT sinuses showed extensive mucosal thickening. Culture from his maxillary sinus and sputum grew pan-sensitive pseudomonas aeruginosa. His bronchial washing grew Methicillin-sensitive Staphylococcus aureus and Mycobacterium abscesses. Physical exam was consistent with chronic sinopulmonary infection, no digit clubbing. His comprehensive immunological deficiency workup was unrevealing. However, his sweat test showed chloride level of 68 to 73 mmol/L. In addition, his genetic test for CFTR showed compound heterozygote for the p.F508del pathogenic variant and [5T; 12TG] allele. The patient was infertile, although no absent vas deferens. There was no family history of CF. Patient was subsequently diagnosed with CF based upon combination of clinical characteristics and laboratory studies. Discussion Patients with CF may present at adult age in a diversity of clinical disciplines. Our case is a reminder that CF should be considered in differential diagnosis of bronchiectasis, even in elderly population.