Snapshot A 25-year-old cystic fibrosis patient presents to his pulmonologist complaining of chronic, frequent coughing productive of yellow and green sputum. He recently recovered from a Pseudomonas spp. pneumonia requiring hospitalization. Introduction Disease caused by multiple cycles of infection and subsequent inflammation in the bronchi and bronchioles that results in permanent fibrosis, remodeling, and dilation of the middle airways Associated with history of chronic infection TB, Pseudomonas spp, Haemophilus spp localized airway obstruction tumor inhaled foreign object reactive airway disease (i.e. asthma) hypersensitivity (i.e. allergic bronchopulmonary aspergillosis) aspiration cystic fibrosis autoimmune disease (i.e. RA, SLE) irritable bowel disease immunodeficiency Presentation Symptoms chronic, productive cough purulent, yellow or green sputum hemoptysis Physical exam rales wheezes rhonchi purulent mucus foul breath Evaluation CXR increased bronchovascular markings tram-track lines parallel lines outlining the dilated bronchi secondary to chronic, peribronchial inflammation and fibrosis honeycombing CT thickened bronchiole walls with dilated airways ballooned cysts at the distal end of the bronchi PFTs decreased FEV1 / FVC ratio Differential Chronic bronchitis, emphysema, cystic fibrosis, and asthma Treatment Non-operative antibiotics indictaed for bacterial infections supplemental O2 indicated when SaO2 <90% inhaled corticosteroids indicated as adjunct to supplemental O2 Operative lobectomy indicated in severe, localized disease lung transplantation should be considered in terminal cases Prognosis, Prevention, and Complications Prognosis ranges widely depending on comorbidities chronic fibrosis is irreversible and can result in severe disease necessitating transplant Prevention early and aggressive treatment of acute lung infections may prevent fibrosis careful monitoring of chronic disease patients for signs of infection/inflammation may slow progression of disease Complications may be so severe that lung transplant is only available option for survival