A rare inflammatory pulmonary disorder. Almost always seen in current or former heavy smokers. Often presents with shortness of breath and chronic cough. Other signs and symptoms include wheezing, inspiratory crackles, exertional dyspnea, and rarely, digital clubbing. Commonly presents in the fourth or fifth decade of life.

Management includes smoking cessation (with mixed results), oxygen supplementation, patient education, and exercise focused on pulmonary rehabilitation, as in treatment of chronic obstructive pulmonary disease. See nicotine dependence for information on smoking cessation. Vaccinations are recommended to prevent viral and bacterial infections, particularly influenza and pneumonia.