Familial Idiopathic Pulmonary Fibrosis

OBJECTIVE: The purpose of this study was to assess the high-resolution computed tomography (CT) findings of familial idiopathic pulmonary fibrosis (IPF). METHODS: High-resolution CT scans obtained in 9 consecutive patients with biopsy-proven familial IPF were reviewed. The presence, distribution, and extent of various CT findings were evaluated. RESULTS: All 9 patients had ground-glass opacities, intralobular reticular opacities, and irregular thickening of the interlobular septa; 7 (78%) had traction bronchiectasis, 7 (78%) had small foci of consolidation; and 3 (33%) had honeycombing. The abnormalities involved mainly the lower lung zones in 6 patients and the upper lung zones in 2 patients and had no zonal predominance in 1 patient. Follow-up CT showed increased extent of disease in 8 patients. CONCLUSIONS: The high-resolution CT findings of familial IPF resemble those of nonfamilial IPF. Familial IPF has a lower prevalence of honeycombing and a lower prevalence of predominant lower lung zone distribution than nonfamilial IPF, however.