title: Comparisons of Prognosis between Surgically and Clinically Diagnosed Idiopathic Pulmonary Fibrosis Using Gap Model: A Korean National Cohort Study.
abstract: Although a multidisciplinary approach has become an important criterion for an idiopathic pulmonary fibrosis (IPF) diagnosis, lung biopsies remain crucial. However, the prognosis of patients with surgically diagnosed IPF (sIPF) is uncertain. We aimed to investigate the prognosis of patients with clinically diagnosed IPF (cIPF) and sIPF. In this retrospective observational study, the Korean Interstitial Lung Disease Study Group conducted a national survey to evaluate the clinical, physiological, radiological, and survival characteristics of patients with IPF from January 1, 2003 to December 31, 2007. Patients were recruited from 54 universities and teaching hospitals across the Republic of Korea. IPF diagnoses were established according to the 2002 American Thoracic Society (ATS)/European Respiratory Society criteria (ERS) guideline. A total of 1685 patients with IPF (1027 cIPF and 658 sIPF) were enrolled. Patients with sIPF were significantly younger, predominantly female, and nonsmokers (all P < 0.001). sIPF group had significantly better initial pulmonary function. The proportion of computed tomography-based honeycomb findings of patients with cIPF was higher than in those with sIPF (P < 0.001). A Kaplan-Meier analysis showed that the sIPF group had a better prognosis (P = 0.001). A survival analysis showed that age, pulmonary function parameters, pulmonary oxygen tension, honeycombing change, and combined lung cancer had a significant influence on patient prognosis. However, there was no significant difference in prognosis between the cIPF and sIPF groups after adjusting for GAP (gender, age, physiology) stage. The patients with sIPF had better clinical features than those with cIPF. However, after adjusting for GAP stage, the sIPF group showed similar prognoses as the cIPF group. This study showed that after adjusting for GAP stage, the prognosis of patients with IPF is the same regardless of the diagnostic method used.
authors.affiliation: From the Department of Internal Medicine, Division of Pulmonology, Severance Hospital, Institute of Chest Diseases, Yonsei University College of Medicine, Seoul, Korea (SHL, SYK, MSP); Division of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center (DSK, JWS); Department of Internal Medicine and Lung Institute, Division of Pulmonary and Critical Care Medicine, Seoul National University College of Medicine (YWK); Division of Pulmonary and Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine (MPC); Department of Internal Medicine, Division of Allergy and Respiratory Medicine, Soonchunhyang University Seoul Hospital (STU); Department of Internal Medicine, Division of Allergy and Respiratory Medicine, Soonchunhyang University Bucheon Hospital (CSP); Department of Internal Medicine, Division of Pulmonology, Gachon University Gil Medical Center (SHJ); Department of Internal Medicine, Division of Pulmonary, Allergy & Critical Care Medicine, Hallym University Kangdong Sacred Heart Hospital (YBP); Department of Internal Medicine, Pulmonary Division, Inha University Hospital (HLL); Department of Internal medicine, Division of Pulmonary Medicine, Chung Ang University College of Medicine (JWS); Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University Anam Hospital, Korea University College of Medicine (EJL); Department of Internal Medicine, Ewha Womans University School of Medicine, Ewha Medical Research Institute (JHL); Department of Internal Medicine, Division of Pulmonary Medicine, Ulsan University Hospital, University of Ulsan College of Medicine (YJ); Department of Internal Medicine, Division of Critical Care and Pulmonary Medicine, Inje University Pusan Paik Hospital (HKL); and Department of Internal Medicine, Division of Allergy and Pulmonology, Bucheon St. Mary's Hospital, The Catholic University of Korea School of Medicine (YHK), Bucheon, Ko
authors.forename: Sang Hoon
authors.initials: SH
authors.lastname: Lee
authors.forename: Song Yee
authors.initials: SY
authors.lastname: Kim
authors.forename: Dong Soon
authors.initials: DS
authors.lastname: Kim
authors.forename: Young Whan
authors.initials: YW
authors.lastname: Kim
authors.forename: Man Pyo
authors.initials: MP
authors.lastname: Chung
authors.forename: Soo Taek
authors.initials: ST
authors.lastname: Uh
authors.forename: Choon Sik
authors.initials: CS
authors.lastname: Park
authors.forename: Sung Hwan
authors.initials: SH
authors.lastname: Jeong
authors.forename: Yong Bum
authors.initials: YB
authors.lastname: Park
authors.forename: Hong Lyeol
authors.initials: HL
authors.lastname: Lee
authors.forename: Jong Wook
authors.initials: JW
authors.lastname: Shin
authors.forename: Eun Joo
authors.initials: EJ
authors.lastname: Lee
authors.forename: Jin Hwa
authors.initials: JH
authors.lastname: Lee
authors.forename: Yangin
authors.initials: Y
authors.lastname: Jegal
authors.forename: Hyun Kyung
authors.initials: HK
authors.lastname: Lee
authors.forename: Yong Hyun
authors.initials: YH
authors.lastname: Kim
authors.forename: Jin Woo
authors.initials: JW
authors.lastname: Song
authors.forename: Moo Suk
authors.initials: MS
authors.lastname: Park
mesh_terms: D000367:Age Factors; D000368:Aged; D001706:Biopsy; D001784:Blood Gas Analysis; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D008168:Lung / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D056910:Republic of Korea; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012737:Sex Factors; D014057:Tomography, X-Ray Computed