Introduction A form of obstructive lung disease Pathophysiology Robust inflammatory response (neutrophil proteases)→ structural damage → mucous stasis → bacterial colonization → continued chronic inflammation characterized bypermanent abnormal dilation of bronchi/bronchioles ↓ of function of cartilage/elastin in conducting airways Causes chronic infection tuberculosis, H. influenzae, and S. aureus atypical mycobacteria bronchial obstruction tumor, foreign body, and lymphadenopathy cystic fibrosis ↑ viscosity of mucus ↑ rate of respiratory infection due to mucus milleu most common cause is P. aeruginosa ↓ ciliary function primary ciliary dyskinesia (Kartagener syndrome) genetic disease resulting in cilia without dyein arm immunodeficiency hypogammaglobulinemia and HIV allergic bronchopulmonary aspergillosis (ABPA) autoimmune RA and Sjogren's syndrome α1-antitrypsin deficiency Presentation Symptoms large amount of purulent sputum recurrent infections hemoptysis Evaluation CXR "tram-track" lung markings due to bronchi extending to periphery CT thickened bronchial walls with dilated airways Treatment Pharmacologic airway clearance with nebulization (saline and hypertonic saline), postural drainage, percussion, and positive expiratory pressure device azithromycin has been shown to decrease exacerbation rates aggressive antibiotics for infections attempts should be made to eradicate P. aeruginosa or MRSA Surgical localized disease can be treated with lobectomy or segmentectomy lung transplant rare other than CF but could be considered if severe
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