Correct!
5. All of the above
Desquamative interstitial pneumonia or pneumonitis (DIP) was first described by Liebow in 1965 (2). It is the rarest of the idiopathic interstitial lung diseases. A characteristic feature is pigment in intraalveolar macrophages. There is a strong association with cigarette smoking with initial reports of up to 90% of patients being current or former smokers. Recent studies suggest a somewhat smaller percentage are smokers of 60 to 87%.
In addition to cigarette smoking there are a number of other associations with DIP (Table 1) (2).
Table 1. Diseases association with DIP
Some patients with diagnosis of DIP have subsequent CT changes that progress to fibrotic NSIP pattern (2). One report of a surgical lung biopsy showing DIP, but subsequent explanted lung showed NSIP. Prognosis seems better in DIP than NSIP, suggesting some differences.
Smoking cessation is critical and may lead to significant clinical improvement (2). Corticosteroid therapy is usually effective, but not all patients respond, particularly if there is continued smoking.
After discussing his diagnosis of DIP, our patient wanted to try smoking cessation but continue with his current low dose of prednisone. The plan will be for close follow up.
References