Treatment revolves around smoking cessation and oftentimes this is the only intervention needed to stabilize symptoms. For patients with progressive or symptomatic disease, corticosteroids are often prescribed, although there are neither controlled treatment trials nor evidence of efficacy beyond anecdotal reports. Although corticosteroids may possibly have a role in the early, nodular stage of the disease, it is exceedingly unlikely that they are efficacious with advanced cystic disease. Other immunosuppressive agents have been used in case reports and series, including vinblastine, methotrexate, cyclophosphamide, etoposide, and chlorodeoxyadenosine. A significant improvement in cystic lung changes, dyspnea, and lung function has been seen with the use of chlorodeoxyadenosine, a purine nucleoside analog. Clinicians should also be aware of the increased risk of pneumothorax in this patient population. Conservative therapy may be used to treat small pneumothoraces, but pleurodesis should be considered for recurrent events or large pneumothoraces. Patients should also be screened for pulmonary hypertension, which becomes increasingly common as the disease progresses and is associated with a poorer prognosis.