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 17-month-old girl presented to the out-patient clinic at our medical center with a 30-day history of worsening cough. Her older sister (Case 3) had been admitted to the intensive care unit at the Asan Medical Center with respiratory failure 1 month previously, and her mother (Case 4) had had similar symptoms during the previous 45 days. On physical examination, the patient was apyrexial, tachycardic, and tachypneic (respiratory rate, 40/min). Her WBC count was 21,900/μL, with 48% neutrophils and 43% lymphocytes. Blood, sputum, and BAL fluid cultures were negative for bacteria, viruses and fungi. However, multiplex RT-PCR of nasopharyngeal aspirates revealed the presence of rhinovirus. Radiography and a CT scan of the chest showed the presence of centrilobular ground-glass opacities and septal thickening in both lungs. She was treated with steroids, cyclophosphamide, and hydroxychloroquine for 2 weeks. She showed significant clinical improvement, and repeat chest radiography 2 weeks after the initiation of treatment revealed that the ground-glass opacities, septal thickening, and consolidation had decreased. After discharge, she did not require oxygen, and her daily activities were not restricted, although she continued to experience dyspnea on exertion.