Non―Cystic Fibrosis Bronchiectasis

• Published in: American journal of respiratory and critical care medicine 2013-09, Vol.188 (6), p.647-656
• Main Authors: MCSHANE, Pamela J, NAURECKAS, Edward T, TINO, Gregory, STREK, Mary E
• Format: Article
• Language: English
• Subjects: Adrenal Cortex Hormones - therapeutic use; Airway management; Airway Management - methods; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Anti-Bacterial Agents - therapeutic use; Anti-Inflammatory Agents - therapeutic use; Biological and medical sciences; Bronchi - physiopathology; Bronchi - surgery; Bronchiectasis - diagnosis; Bronchiectasis - physiopathology; Bronchiectasis - therapy; Bronchodilator Agents - therapeutic use; Chronic obstructive pulmonary disease; Cystic fibrosis; Errors of metabolism; Etiology; Exercise Therapy - methods; General aspects; Graft versus host disease; Human infectious diseases. Experimental studies and models; Humans; Infections; Infectious diseases; Inflammation; Intensive care medicine; Macrolides - therapeutic use; Medical sciences; Metabolic diseases; Miscellaneous hereditary metabolic disorders; Mortality; Pneumology; Respiratory system : syndromes and miscellaneous diseases; Tomography
• ISSN: 1073-449X; 1535-4970
• DOI: 10.1164/rccm.201303-0411CI

There is renewed interest in non-cystic fibrosis bronchiectasis, which is a cause of significant morbidity in adults and can be diagnosed by high-resolution chest computed tomography scan. No longer mainly a complication after pulmonary infection with Mycobacterium tuberculosis, diverse disease processes and mechanisms have been demonstrated to result in the chronic cough, purulent sputum production, and airway dilation that characterize this disease. Improved understanding of the role of mucus stasis in causing bacterial colonization has led to increased emphasis on the use of therapies that enhance airway clearance. Inhalational antibiotics reduce the bacterial burden associated with a worse outcome. Low-dose, chronic macrolide therapy has been shown to decrease exacerbation frequency and airway inflammation. For the first time, a number of therapies for non-cystic fibrosis bronchiectasis are undergoing testing in clinical research trials designed specifically for this population. This concise clinical review focuses on the major etiologies, diagnostic testing, microbiology, and management of patients with adult non-cystic fibrosis bronchiectasis. Systematic evaluation identifies a specific cause in the majority of patients and may affect subsequent treatment. We outline current therapies and review the data that support their use.