Pathological characteristics in idiopathic nonspecific interstitial pneumonia with emphysema and pulmonary hypertension

A 75-year-old man was admitted to our hospital complaining of a 4-year history of persistent dry cough and progressive dyspnea on exertion. Chest computed tomography images revealed diffuse reticular opacities and traction bronchiectasis in the bilateral lower lobes and emphysema predominantly in the upper lobes. He was treated with inhaled N-acetylcystein therapy, oral corticosteroids, and pirfenidone in addition to oxygen administration. However, his symptoms and oxygenation gradually deteriorated. In addition, echocardiography showed that estimated pulmonary arterial pressure was 109 mm Hg, sildenafil was started. Twenty months later, he suddenly died of decompensated right heart failure. The autopsied lungs demonstrated a diffuse fibrotic nonspecific interstitial pneumonia (NSIP) pattern with emphysema (combined pulmonary fibrosis and emphysema) and widespread severe intimal and medial thickening ranging from proximal elastic to distal muscular pulmonary arteries. To our knowledge, little has been reported on clinicopathological characteristics of idiopathic NSIP associated with emphysema and severe pulmonary hypertension.