| Autor: |
Steele MP; Department of Medicine., Peljto AL; Department of Medicine., Mathai SK; Center for Advanced Heart and Lung Disease, Baylor University Medical Center at Dallas, Dallas, Texas., Humphries S; Department of Radiology., Bang TJ; Department of Radiology, and., Oh A; Department of Radiology., Teague S; Department of Radiology., Cicchetti G; Department of Diagnostic Imaging, Oncological Radiotherapy, and Hematology, Fondazione Policlinico University Gemelli, Rome, Italy., Sigakis C; Department of Regional Radiology, Cleveland Clinic Imaging Institute, Cleveland, Ohio; and., Kropski JA; Department of Medicine, Vanderbilt University, Nashville, Tennessee., Loyd JE; Department of Medicine, Vanderbilt University, Nashville, Tennessee., Blackwell TS; Department of Medicine, Vanderbilt University, Nashville, Tennessee., Brown KK; Department of Medicine, and., Schwarz MI; Department of Medicine., Warren RA; Department of Medicine., Powers J; Department of Medicine., Walts AD; Department of Medicine., Markin C; Department of Medicine, Vanderbilt University, Nashville, Tennessee., Fingerlin TE; Center for Genes, Environment, and Health, National Jewish Health, Denver, Colorado., Yang IV; Department of Medicine., Lynch DA; Department of Radiology., Lee JS; Department of Medicine., Schwartz DA; Department of Medicine.; Department of Microbiology and Immunology, University of Colorado School of Medicine, Aurora, Colorado. |
| Abstrakt: |
Rationale: Relatives of patients with familial interstitial pneumonia (FIP) are at increased risk for pulmonary fibrosis and develop preclinical pulmonary fibrosis (PrePF). Objectives: We defined the incidence and progression of new-onset PrePF and its relationship to survival among first-degree relatives of families with FIP. Methods: This is a cohort study of family members with FIP who were initially screened with a health questionnaire and chest high-resolution computed tomography (HRCT) scan, and approximately 4 years later, the evaluation was repeated. A total of 493 asymptomatic first-degree relatives of patients with FIP were evaluated at baseline, and 296 (60%) of the original subjects participated in the subsequent evaluation. Measurements and Main Results: The median interval between HRCTs was 3.9 years (interquartile range, 3.5-4.4 yr). A total of 252 subjects who agreed to repeat evaluation were originally determined not to have PrePF at baseline; 16 developed PrePF. A conservative estimate of the annual incidence of PrePF is 1,023 per 100,000 person-years (95% confidence interval, 511-1,831 per 100,000 person-years). Of 44 subjects with PrePF at baseline, 38.4% subjects had worsening dyspnea compared with 15.4% of those without PrePF ( P = 0.002). Usual interstitial pneumonia by HRCT ( P < 0.0002) and baseline quantitative fibrosis score ( P < 0.001) are also associated with worsening dyspnea. PrePF at the initial screen is associated with decreased survival ( P < 0.001). Conclusions: The incidence of PrePF in this at-risk population is at least 100-fold higher than that reported for sporadic idiopathic pulmonary fibrosis (IPF). Although PrePF and IPF represent distinct entities, our study demonstrates that PrePF, like IPF, is progressive and associated with decreased survival. |
