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 30-year-old non-smoking woman, who was the mother of Cases 3 and 5, was admitted to the Department of Internal Medicine at the Asan Medical Center with a 1-month history of cough and a 10-day history of dyspnea on exertion. On physical examination, she was apyrexial and tachypneic (respiratory rate, 44/ min), and crackles were audible over the right lung on auscultation. No hypoxemia was detected by ABGA. Her leukocyte count was 14,000/μL, with 72% neutrophils and 20% lymphocytes. The ESR was 38 mm/hr; and the CRP was 1.92 mg/dL. Laboratory tests for collagen vascular disease and vasculitis were negative. Blood, sputum, and BAL fluid cultures were negative for bacteria, viruses, and fungi. Pulmonary function tests showed a mild restrictive defect. Radiography and a CT scan of the chest revealed the presence of multifocal patchy consolidation with ground-glass opacities and tiny nodular lesions in both lungs. The patient was treated with steroid pulse therapy, followed by oral steroids, azathioprine, and antibiotics. The dyspnea gradually decreased, and she was discharged after 2 weeks of treatment. A repeat CT scan of the chest 12 days postdischarge showed an increase in the extent of ground-glass opacities and tiny nodules in both lungs. She was readmitted 14 days post-discharge with increased dyspnea. Cyclosporine was added to her existing treatment regimen of steroids and azathioprine. Transbronchial biopsy showed a nonspecific subacute lung injury pattern, including focal-organizing pneumonia and mild interstitial chronic inflammation. A follow-up chest CT scan 4 weeks after readmission revealed mild resolution of the abnormalities in both lungs. After discharge, her daily activities were limited by dyspnea, and she continued to experience dyspnea at rest.