Two Series of Familial Cases With Unclassified Interstitial Pneumonia With Fibrosis

Lee, Eun; Seo, Ju-Hee; Kim, Hyoung-Young; Yu, Jinho; Song, Jin Woo; Park, Young Soo; Jang, Se-Jin; Do, Kyung-Hyun; Kwon, Jiwon; Park, Sung-woo; Park, Jeong-hwan; Hong, Soo-Jong

A previously healthy 47-year-old non-smoking male patient presented with a 2-day history of cough, mucoid sputum, and chills. His son was hospitalized with the same symptoms at the same time. On physical examination, he was apyrexial, tachycardic, and tachypneic, and rales were audible over the left lung field on auscultation. Chest radiography showed diffuse hazy patches in the left lung, and a chest CT scan revealed the presence of multifocal consolidation and diffuse centrinodular ground-glass opacities in both lungs. Laboratory findings included a normal WBC count, an elevated erythrocyte sedimentation rate (ESR) of 42 mm/hr, an elevated C-reactive protein (CRP) level of 5.19 mg/dL, and a normal ABGA. Laboratory tests for collagen disease and vasculitis were negative. Blood, sputum, and BAL fluid cultures were negative for bacteria, viruses, and fungi. Lung biopsy, performed on day 11 of admission, showed histological features of fibroproliferative DAD with diffuse interstitial fibrosis and relative sparing of the peripheral lobular air spaces. The patient required mechanical ventilation for a total of 36 days. He was administered broad-spectrum antibiotics and high-dose corticosteroids. On discharge, he still experienced dyspnea on exertion and required intermittent oxygen at night and on exertion. At the 1-year follow-up, radiography and a CT scan revealed a decrease in the degree of bronchial dilatation and peribronchial consolidation; the patient still complained of dyspnea on exertion.