Smoking-related interstitial lung disease in adults

Respiratory bronchiolitis–interstitial lung disease, Langerhans cell histiocytosis and desquamative interstitial pneumonia are strongly associated with smoking.

Smoking cessation is the principal recommendation for management because quitting smoking can lead to clinical and physiological improvements. Corticosteroids can be used in refractory or progressive cases, although evidence for their use is limited. Lung transplantation may need to be considered in advanced cases.

Many patients with idiopathic pulmonary fibrosis have a history of smoking. Patients with combined pulmonary fibrosis and emphysema (CPFE), characterised by upper lobe emphysema and lower lobe interstitial fibrosis, have a high incidence of associated pulmonary hypertension and a poor prognosis.