The Elusive Goal of Therapy for Usual Interstitial Pneumonia

Abstract

Idiopathic pulmonary fibrosis, defined pathologically as usual interstitial pneumonia, is a fatal disease that occurs most commonly among persons who are 60 years of age or older and in otherwise good health. Usual interstitial pneumonia progressively impairs breathing, but because its onset is insidious, patients are frequently treated for other diseases for many months. By the time the diagnosis is established, pulmonary function has often substantially deteriorated.¹ Several entities fall under the wide umbrella of chronic interstitial pneumonias,² including usual interstitial pneumonia, nonspecific interstitial pneumonitis, desquamative interstitial pneumonia, acute interstitial pneumonia, respiratory bronchiolitis–associated interstitial lung disease, and cryptogenic organizing pneumonia. . . .