An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management
Ganesh Raghu
(1)
,
Harold R. Collard
(2)
,
Jim J. Egan
(3)
,
Fernando J. Martinez
(4)
,
Juergen Behr
(5)
,
Kevin K. Brown
(6)
,
Thomas V. Colby
(7)
,
Jean-François Cordier
(8)
,
Kevin R. Flaherty
(4)
,
Joseph A. Lasky
(9)
,
David A. Lynch
(10, 11)
,
Jay H. Ryu
(7)
,
Jeffrey J. Swigris
(6)
,
Athol U. Wells
(12, 13)
,
Julio Ancochea
(14)
,
Demosthenes Bouros
(15)
,
Carlos Carvalho
(16)
,
Ulrich Costabel
,
Masahito Ebina
(17)
,
David M. Hansell
(18)
,
Takeshi Johkoh
(19)
,
Dong Soon Kim
(20)
,
Talmadge E. King, Jr
(2)
,
Yasuhiro Kondoh
(21)
,
Jeffrey Myers
(4)
,
Nestor L. Muller
(22)
,
Andrew G. Nicholson
(23)
,
Luca Richeldi
(24)
,
Moises Selman
(25)
,
Rosalind F. Dudden
(6)
,
Barbara S. Griss
(6)
,
Shandra L. Protzko
(6)
,
Holger J. Schünemann
(26, 27)
1
University of Washington [Seattle]
2 UC San Francisco - University of California [San Francisco]
3 The Mater Hospital - Mater Misericordiae University Hospital
4 University of Michigan [Ann Arbor]
5 LMU - Ludwig-Maximilians University [Munich]
6 National Jewish Health
7 Mayo Clinic
8 Rétrovirus et Pathologie Comparée
9 Tulane University
10 University of Colorado Cancer Center [Aurora, CO, USA]
11 University of Colorado [Boulder]
12 Royal Brompton Hospital
13 Imperial College London
14 Hospital Universitario de La Princesa
15 University Hospital of Alexandroupolis
16 USP - Universidade de São Paulo = University of São Paulo
17 Tohoku University [Sendai]
18 Royal Brompton and Harefield NHS Foundation Trust
19 Osaka University [Osaka]
20 University of Ulsan
21 Department of Respiratory Medicine and Allergy,
22 UBC - University of British Columbia
23 Department of Histopathology
24 UNIMORE - Università degli Studi di Modena e Reggio Emilia = University of Modena and Reggio Emilia
25 Instituto Nacional de Enfermedades Respiratorias
26 Department of Clinical Epidemiology and Biostatistics and Medicine
27 Department of Medicine
2 UC San Francisco - University of California [San Francisco]
3 The Mater Hospital - Mater Misericordiae University Hospital
4 University of Michigan [Ann Arbor]
5 LMU - Ludwig-Maximilians University [Munich]
6 National Jewish Health
7 Mayo Clinic
8 Rétrovirus et Pathologie Comparée
9 Tulane University
10 University of Colorado Cancer Center [Aurora, CO, USA]
11 University of Colorado [Boulder]
12 Royal Brompton Hospital
13 Imperial College London
14 Hospital Universitario de La Princesa
15 University Hospital of Alexandroupolis
16 USP - Universidade de São Paulo = University of São Paulo
17 Tohoku University [Sendai]
18 Royal Brompton and Harefield NHS Foundation Trust
19 Osaka University [Osaka]
20 University of Ulsan
21 Department of Respiratory Medicine and Allergy,
22 UBC - University of British Columbia
23 Department of Histopathology
24 UNIMORE - Università degli Studi di Modena e Reggio Emilia = University of Modena and Reggio Emilia
25 Instituto Nacional de Enfermedades Respiratorias
26 Department of Clinical Epidemiology and Biostatistics and Medicine
27 Department of Medicine
Abstract
This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format. For each diagnosis and treatment question, the committee graded the quality of the evidence available (high, moderate, low, or very low), and made a recommendation (yes or no, strong or weak). Recommendations were based on majority vote. It is emphasized that clinicians must spend adequate time with patients to discuss patients' values and preferences and decide on the appropriate course of action.