Bronchiectasis and Mycobacterium Avium Complex(MAC) infection is associated with Hereditary Disorders of Connective Tissue. B. Griswold1, L. Sloper1, C.A. Francomano2, N.B. McDonnell1 1) LCI, NIA, Baltimore, MD; 2) GBMC, Baltimore, MD.
Bronchiectasis is an abnormal stretching and enlarging of the respiratory passages and may predispose the patients to respiratory infections. A previous study noted a relationship between bronchiectasis and the presence of scoliosis, however did not discuss the role of heritable disorders of connective tissue (HDCT). In a cohort of 95 patients with Ehlers-Danlos syndrome (EDS) enrolled at a natural history study of HDCT at the National Institutes of Health, we identified four patients with bronchiectasis and infection with Mycobacterium avium complex (MAC) in the setting of intact immune function. The organism was identified by specimens obtained during bronchoscopy. MAC consists of two speciesM. avium and M. intracellulare, and is found in water supplies, house dust, soil, farm animals, birds, and cigarette components. It rarely causes pathology in the immunocompetent host. Two of the subjects were women, ages 39 and 50, with hypermobility type of EDS and Marfanoid body habitus, mild scoliosis and dolicocephaly. The third patient was a 50 year old woman with classical EDS, with atrophic scars, doughy skin and joint hypermobility and no evidence of scoliosis. The fourth patient was a teenage boy with hypermobile EDS, stretchy skin without atrophic scars. He had congenital stenosis of the lumbar spine, however, did not have scoliosis. Two patients were diagnosed with bronchiectasis and MAC prior to enrollment in the study, and the others developed the infection during the follow-up period.