TY - JOUR
T1 - Incidence and Progression of Fibrotic Lung Disease in an At-Risk Cohort
AU - Steele, Mark P.
AU - Peljto, Anna L.
AU - Mathai, Susan K.
AU - Humphries, Stephen
AU - Bang, Tami J.
AU - Oh, Andrea
AU - Teague, Shawn
AU - Cicchetti, Giuseppe
AU - Sigakis, Christopher
AU - Kropski, Jonathan A.
AU - Loyd, James E.
AU - Blackwell, Timothy S.
AU - Brown, Kevin K.
AU - Schwarz, Marvin I.
AU - Warren, Rachel A.
AU - Powers, Julia
AU - Walts, Avram D.
AU - Markin, Cheryl
AU - Fingerlin, Tasha E.
AU - Yang, Ivana V.
AU - Lynch, David A.
AU - Lee, Joyce S.
AU - Schwartz, David A.
PY - 2023/3/1
Y1 - 2023/3/1
N2 - 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.
AB - 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.
KW - familial idiopathic pulmonary fibrosis
KW - idiopathic pulmonary fibrosis
KW - pulmonary fibrosis
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UR - http://www.scopus.com/inward/citedby.url?scp=85146872038&partnerID=8YFLogxK
U2 - 10.1164/rccm.202206-1075OC
DO - 10.1164/rccm.202206-1075OC
M3 - Article
C2 - 36094461
AN - SCOPUS:85146872038
SN - 1073-449X
VL - 207
SP - 587
EP - 593
JO - American journal of respiratory and critical care medicine
JF - American journal of respiratory and critical care medicine
IS - 5
ER -