Familial idiopathic pulmonary fibrosis: serial high-resolution computed tomography findings in 9 patients.
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.
Nishiyama
Osamu
O
Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan. nishiyama_o@yahoo.co.jp
Taniguchi
Hiroyuki
H
Kondoh
Yasuhiro
Y
Kimura
Tomoki
T
Katoh
Toshiyuki
T
Oishi
Takashi
T
Matsumoto
Shuichi
S
Yokoi
Toyoharu
T
Takagi
Kenzo
K
Shimokata
Kaoru
K
Johkoh
Takeshi
T
Müller
Nestor L
NL
D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001987:Bronchiectasis / Q000000981:diagnostic imaging; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008168:Lung / Q000000981:diagnostic imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000235:genetics*; D018709:Statistics, Nonparametric; D015996:Survival Rate; D014057:Tomography, X-Ray Computed / Q000379:methods*; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone