Interstitial lung disease (ILD) is found in up to 30% of patients with rheumatoid arthritis (RA) and is clinically manifest in 5 to 10%, resulting in significant morbidity and mortality. The most frequent histopathological forms are usual interstitial pneumonia and nonspecific interstitial pneumonia. Another recently described presentation is combined pulmonary fibrosis and emphysema. Similarly to idiopathic pulmonary fibrosis, acute exacerbation of ILD may occur in RA and is associated with severe prognosis. Smoking is a known risk factor of RA and may also play a role in the pathogenesis of RA-associated ILD, in combination with genetic and immunologic mechanisms. Several treatments of RA may also lead to drug-induced ILD.