Am. J. Respir. Crit. Care Med., Volume 162, Number 4, October 2000, 1277-1284

An Investigation into Causative Factors in Patients with Bronchiectasis

MARK C. PASTEUR, SHARON M. HELLIWELL, SALLY J. HOUGHTON, SUSANNAH C. WEBB, JULIET E. FOWERAKER, RICHARD A. COULDEN, CHRISTOPHER D. FLOWER, DIANA BILTON, and MARY T. KEOGAN

Lung Defence Unit, Department of Immunology, Department of Microbiology, and Department of Radiology, Papworth Hospital, Cambridge, United Kingdom

Bronchiectasis is a pathologic description of lung damage characterized by inflamed and dilated thick-walled bronchi. These findings may result from a number of possible causes and these may influence treatment and prognosis. The aim of this study was to determine causative factors in 150 adults with bronchiectasis (56 male, 94 female) identified using high-resolution computerized tomography. Relevant factors were identified in the clinical history; cystic fibrosis gene mutation analysis was performed; humoral immune defects were determined by measuring immunoglobulins, IgG subclasses and functional response to Pneumovax II vaccine; assessment was made of neutrophil function (respiratory burst, adhesion molecule expression, and chemotaxis); ciliary function was observed and those likely to have allergic bronchopulmonary aspergillosis (ABPA) were identified. Causes identified were: immune defects (12 cases), cystic fibrosis (), Young's syndrome (), ciliary dysfunction (), aspiration (), panbronchiolitis (), congenital defect (), ABPA (), rheumatoid arthritis (), and early childhood pneumonia, pertussis, or measles (). Intensive investigation of this population of patients with bronchiectasis led to identification of one or more causative factor in 47% of cases. In 22 patients (15%), the cause identified had implications for prognosis and treatment.

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