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Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis.
Hunninghake, Gary M; Quesada-Arias, Luisa D; Carmichael, Nikkola E; Martinez Manzano, Jose M; Poli De Frías, Sergio; Baumgartner, Maura Alvarez; DiGianni, Lisa; Gampala-Sagar, Shannon N; Leone, Dominick A; Gulati, Swati; El-Chemaly, Souheil; Goldberg, Hilary J; Putman, Rachel K; Hatabu, Hiroto; Raby, Benjamin A; Rosas, Ivan O.
Afiliación
  • Hunninghake GM; Pulmonary and Critical Care Division.
  • Quesada-Arias LD; Center for Pulmonary Functional Imaging.
  • Carmichael NE; Pulmonary and Critical Care Division.
  • Martinez Manzano JM; Division of Pulmonary Medicine, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; and.
  • Poli De Frías S; Pulmonary and Critical Care Division.
  • Baumgartner MA; Pulmonary and Critical Care Division.
  • DiGianni L; Pulmonary and Critical Care Division.
  • Gampala-Sagar SN; Division of Pulmonary Medicine, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts; and.
  • Leone DA; Channing Division of Network Medicine, and.
  • Gulati S; Channing Division of Network Medicine, and.
  • El-Chemaly S; Pulmonary and Critical Care Division.
  • Goldberg HJ; Pulmonary and Critical Care Division.
  • Putman RK; Pulmonary and Critical Care Division.
  • Hatabu H; Pulmonary and Critical Care Division.
  • Raby BA; Center for Pulmonary Functional Imaging.
  • Rosas IO; Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
Am J Respir Crit Care Med ; 201(10): 1240-1248, 2020 05 15.
Article en En | MEDLINE | ID: mdl-32011908
Rationale: Although relatives of patients with familial pulmonary fibrosis (FPF) are at an increased risk for interstitial lung disease (ILD), the risk among relatives of sporadic idiopathic pulmonary fibrosis (IPF) is not known.Objectives: To identify the prevalence of interstitial lung abnormalities (ILA) and ILD among relatives of patients with FPF and sporadic IPF.Methods: Undiagnosed first-degree relatives of patients with pulmonary fibrosis (PF) consented to participate in a screening study that included the completion of questionnaires, pulmonary function testing, chest computed tomography, a blood sample collection for immunophenotyping, telomere length assessments, and genetic testing.Measurements and Main Results: Of the 105 relatives in the study, 33 (31%) had ILA, whereas 72 (69%) were either indeterminate or had no ILA. Of the 33 relatives with ILA, 19 (58%) had further evidence for ILD (defined by the combination of imaging findings and pulmonary function testing decrements). There was no evidence in multivariable analyses that the prevalence of either ILA or ILD differed between the 46 relatives with FPF and the 59 relatives with sporadic IPF. Relatives with decrements in either total lung or diffusion capacity had a greater than 9-fold increase in their odds of having ILA (odds ratio, 9.6; 95% confidence interval, 3.1-29.8; P < 0.001).Conclusions: An undiagnosed form of ILD may be present in greater than 1 in 6 older first-degree relatives of patients with PF. First-degree relatives of patients with both familial and sporadic IPF appear to be at similar risk. Our findings suggest that screening for PF in relatives might be warranted.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Familia / Enfermedades Pulmonares Intersticiales / Fibrosis Pulmonar Idiopática / Pulmón Tipo de estudio: Prevalence_studies / Prognostic_studies / Risk_factors_studies Límite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Am J Respir Crit Care Med Asunto de la revista: TERAPIA INTENSIVA Año: 2020 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Familia / Enfermedades Pulmonares Intersticiales / Fibrosis Pulmonar Idiopática / Pulmón Tipo de estudio: Prevalence_studies / Prognostic_studies / Risk_factors_studies Límite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Am J Respir Crit Care Med Asunto de la revista: TERAPIA INTENSIVA Año: 2020 Tipo del documento: Article Pais de publicación: Estados Unidos