Desquamative interstitial pneumonia (DIP) is a rare pattern of diffuse parenchymal lung disease known as one of the idiopathic interstitial pneumonias and is considered to be a smoking- or dust inhalation-related interstitial pneumonia in the majority of cases. This report presents the first case of DIP in which the pulmonary manifestation preceded the onset of rheumatoid arthritis. This case and our review of twenty-four DIP cases (nineteen cases previously-reported from Japan, plus five cases in our departments) indicate the possibility that the DIP pattern is an additional form of diffuse interstitial pneumonia that may develop in association with autoimmune diseases.