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Ats-ers Iip Statement Ajrccm 2013

Ats-ers Iip Statement Ajrccm 2013

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 American Thoracic Society Documents
An Official American Thoracic Society/EuropeanRespiratory Society Statement: Update of theInternational Multidisciplinary Classification of theIdiopathic Interstitial Pneumonias
William D. Travis, Ulrich Costabel, David M. Hansell, Talmadge E. King, Jr., David A. Lynch, Andrew G. Nicholson,Christopher J. Ryerson, Jay H. Ryu, Moise´s Selman, Athol U. Wells, Jurgen Behr, Demosthenes Bouros,Kevin K. Brown, Thomas V. Colby, Harold R. Collard, Carlos Robalo Cordeiro, Vincent Cottin, Bruno Crestani,Marjolein Drent, Rosalind F. Dudden, Jim Egan, Kevin Flaherty, Cory Hogaboam, Yoshikazu Inoue, Takeshi Johkoh,Dong Soon Kim, Masanori Kitaichi, James Loyd, Fernando J. Martinez, Jeffrey Myers, Shandra Protzko,Ganesh Raghu, Luca Richeldi, Nicola Sverzellati, Jeffrey Swigris, and Dominique Valeyre; on behalf of the ATS/ERSCommittee on Idiopathic Interstitial Pneumonias
T
HIS OFFICIAL STATEMENT OF THE
 A
MERICAN
 T
HORACIC
 S
OCIETY
 (ATS)
 AND THE
 E
UROPEAN
 R
ESPIRATORY
 S
OCIETY
 (ERS)
 WASAPPROVED BY THE
 ATS B
OARD OF
 D
IRECTORS
, J
UNE
 2013,
 AND BY THE
 ERS S
TEERING
 C
OMMITTEE
, M
ARCH
 2013
CONTENTS
Executive SummaryIntroductionMethodsSummary of Major Revisions of the IIP ClassificationGeneral Progress in IIPs since 2002Multidisciplinary ApproachObserver Agreement in Diagnosis of IIPImportant Differential Diagnostic ConsiderationsHypersensitivity PneumonitisCollagen Vascular DiseaseFamilial Interstitial PneumoniaCoexisting PatternsProgress in Specific IIPs since 2002Chronic Fibrosing IIPsSmoking-related IIPsAcute or Subacute IIPsRare IIPsIdiopathic Lymphoid Interstitial PneumoniaIdiopathic Pleuroparenchymal FibroelastosisRare Histologic PatternsAcute Fibrinous and Organizing PneumoniaBronchiolocentric Patterns of Interstitial PneumoniaUnclassifiable IIPClinical Classification of Disease BehaviorBiomarkers
Background 
: In 2002 the American Thoracic Society/European Res-piratory Society (ATS/ERS) classification of idiopathic interstitialpneumonias(IIPs)definedsevenspecificentities,andprovidedstan-dardizedterminologyanddiagnosticcriteria.Inaddition,thehistorical“goldstandardofhistologicdiagnosiswasreplacedbyamultidiscipli-naryapproach.Since2002manypublicationshaveprovidednewinfor-mationaboutIIPs.
Purpose 
: The objective of this statement is to update the 2002 ATS/ERS classification of IIPs.
Methods 
: An international multidisciplinary panel was formed anddeveloped key questions that were addressed through a review of the literature published between 2000 and 2011.
Results 
:SubstantialprogresshasbeenmadeinIIPssincethepreviousclassification. Nonspecific interstitial pneumonia is now better de-fined.Respiratorybronchiolitis–interstitiallungdiseaseisnowcom-monly diagnosed without surgical biopsy. The clinical course of idi-opathicpulmonaryfibrosisandnonspecificinterstitialpneumoniaisrecognized to be heterogeneous. Acute exacerbation of IIPs is nowwell defined. A substantial percentage of patients with IIP are diffi-culttoclassify,oftenduetomixedpatternsoflung injury.Aclassifi-cation based on observed disease behavior is proposed for patientswhoaredifficulttoclassifyorforentitieswithheterogeneityinclin-ical course. A group of rare entities, including pleuroparenchymalfibroelastosisandrarehistologicpatterns,isintroduced.Therapidlyevolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determiningdiagnosis, and potentially prognosisand treatment.
Conclusions 
: This update is a supplement to the previous 2002 IIPclassification document. It outlines advances in the past decadeand potential areas for future investigation.Keywords
: idiopathic interstitial pneumonia; usual interstitial pneumo-nia; nonspecific interstitial pneumonia; respiratory bronchiolitis; desqua-mative interstitial pneumonia; cryptogenic organizing pneumonia; acuteinterstitial pneumonia; lymphoid interstitial pneumonia; pleuroparenchy-mal fibroelastosis; acute fibrinous and organizing pneumonia
EXECUTIVE SUMMARY
There are several specific areas that are given special attention inthis revision of the 2002 American Thoracic Society/EuropeanRespiratorySocietyidiopathicinterstitialpneumonia(IIP)state-ment.1. Idiopathic nonspecific interstitial pneumonia (NSIP) isnow accepted as a specific clinicopathologic entity. It hasbecome evident that clinical progression is highly het-erogeneous, with several studies suggesting that a subsetof patients demonstrate progression to end-stage fibro-sis; criteria to define this group at the time of diagnosiswould be helpful.
This document has an online supplement, which is accessible from this issue’stable of contents at www.atsjournals.org
Am J Respir Crit Care Med Vol 188, Iss. 6, pp 733–748, Sep 15, 2013Copyright
 ª
 2013 by the American Thoracic SocietyDOI: 10.1164/rccm.201308-1483STInternet address: www.atsjournals.org
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2. New information has accumulated on smoking-related inter-stitiallung disease,including patients with combined emphy-sema and interstitial fibrosis. In clinical practice, respiratorybronchiolitis–interstitial lung disease is increasingly diag-nosed without surgical lung biopsy in smokers on the basisof clinical and imaging features (ground-glass opacities andcentrilobular nodules) and bronchoalveolar lavage (smok-er’s macrophages and absence of lymphocytosis).3. The natural progression of idiopathic pulmonary fibrosis(IPF) is acknowledged to be heterogeneous with somepatients remaining stable for prolonged periods, othersshowing more rapid steady progression, and still otherssuccumbing to acute exacerbation.4. Acute exacerbation is better defined and recognized tooccur in chronic fibrosing IIPs (IPF and NSIP).5. Some patients with IIP are difficult to classify, oftenbecause of mixed patterns of lung injury.6. It is recognized that there is a need to provide a clinicalalgorithm for classifying and managing IIP cases. This isparticularly applicable when no biopsy is available andhigh-resolution computed tomography is not diagnostic.7. Pleuroparenchymalfibroelastosisisrecognizedasaspecificrare entity, usually idiopathic. Other less well-defined his-tologic patterns, such as bronchiolocentric inflammationand fibrosis, are also included.8. A rapidly emerging field of molecular markers holdspromise for improving diagnostic approaches. Thesemarkers may also be useful in predicting prognosis andresponse to different therapies. Incorporation of geneticand molecular studies may revolutionize the approachto diagnosis and classification of the IIPs.
INTRODUCTION
The objective of this statement is to update the 2002 AmericanThoracicSociety/EuropeanRespiratorySociety(ATS/ERS)clas-sification of idiopathic interstitial pneumonias (IIPs) (1). Focus isplaced on describing changes to previously described clinical enti-ties, describing new clinical entities, and describing new histologicpatterns. This update is not intended as a stand-alone documentand should be used as a supplement to the original 2002 IIP classi-fication. In 2002, the ATS/ERS IIP classification (1) defined sevendisease categories, and proposed standardized terminology anddiagnostic criteria. In addition, the historical “gold standard” of histologic diagnosis was replaced by a “dynamic integrated ap-proach” using multidisciplinary discussion (MDD). The 2002 IIPclassification was used in 75% (157 of 208) of all clinical publica-tions on the topic of IIPs between 2004 and 2011. The new infor-mation from these publications is incorporated in this update.
METHODS
This project was performed under supervision by the ATS Docu-ments Development and Implementation Committee in collab-oration with the ERS (Table E1 in the online supplement). Aninternational multidisciplinary panel was assembled. The panelconsisted of 34 experts in interstitial lung diseases (19 pulmonol-ogists, 4 radiologists, 5 pathologists, 2 experts in evidence-basedmedicine, and 4 molecular biologists). Several meetings wereheld by members of the international multidisciplinary panel(Table E2), who disclosed conflicts of interest, which were vettedaccording to ATS and ERS policies.Key questions were developed that the committee believedimportant for the classification of IIPs (
 see
 A
PPENDIX 1
 in theonline supplement). A literature search was performed to iden-tify new publications that pertained to these key questions,assisted by two librarians experienced in literature searches forpulmonary diseases. Literature retrieved from Medline searchesbetween 2000 and 2011 was used to produce this statement.The committee was divided into subgroups assigned to spe-cific sections of the document. These subgroups reviewedthe rel-evant literature and produced the first draft of their respectivesections. These sections were compiled by the committee chairand a complete first draft was edited by the writing subcommit-tee. This document was reviewed and edited by all committeemembers before final review by the writing subcommittee.The revised document was approved by all authors.
SUMMARY OF MAJOR REVISIONS OF THEIIP CLASSIFICATION
In the revision of the IIP classification, the main entities are pre-served (Table 1). However, there are several important changes.First,cryptogenic fibrosing alveolitis is removed, leaving idiopathicpulmonary fibrosis (IPF) as the sole clinical termfor this diagnosis.Second,idiopathicnonspecificinterstitialpneumonia(NSIP)isnowaccepted as a distinct clinical entity with removal of the term “pro-visional”(2).Third,majorIIPsaredistinguishedfromrareIIPsandunclassifiable cases. Fourth, rare histologic patterns of acute fibri-nous and organizing pneumonia (AFOP) and interstitial pneumo-nias with a bronchiolocentric distribution arerecognized. Fifth, themajor IIPs are grouped into chronic fibrosing (IPF and NSIP; Fig-ures1and2),smoking-related(respiratorybronchiolitis–interstitiallung disease [RB-ILD] and desquamative interstitial pneumonia[DIP]; Figure 3), and acute/subacute IIPs (cryptogenic organizingpneumonia[COP]andacuteinterstitialpneumonia[AIP];Figure4and Table 2). Sixth, a clinical disease behavior classification is pro-posed. Last, molecular and genetic features are reviewed.
GENERAL PROGRESS IN IIPS SINCE 2002
Multidisciplinary Approach
Theprocessofachievingamultidisciplinarydiagnosisinapatientwith IIP is dynamic, requiring close communication between cli-nician, radiologist, and when appropriate, pathologist (1). Clin-ical data (presentation, exposures, smoking status, associated
TABLE 1. REVISED AMERICAN THORACIC SOCIETY/EUROPEANRESPIRATORY SOCIETY CLASSIFICATION OF IDIOPATHICINTERSTITIAL PNEUMONIAS: MULTIDISCIPLINARY DIAGNOSES
Major idiopathic interstitial pneumoniasIdiopathic pulmonary fibrosisIdiopathic nonspecific interstitial pneumoniaRespiratory bronchiolitis–interstitial lung diseaseDesquamative interstitial pneumoniaCryptogenic organizing pneumonia Acute interstitial pneumoniaRare idiopathic interstitial pneumoniasIdiopathic lymphoid interstitial pneumoniaIdiopathic pleuroparenchymal fibroelastosisUnclassifiable idiopathic interstitial pneumonias**Causes of unclassifiable idiopathic interstitial pneumonia include (
1
) inade-quate clinical, radiologic, or pathologic data and (
2
) major discordance betweenclinical, radiologic, and pathologic findings that may occur in the following situa-tions: (
) previous therapy resulting in substantial alteration of radiologic or histo-logic findings (e.g., biopsy of desquamative interstitial pneumonia after steroidtherapy, which shows only residual nonspecific interstitial pneumonia [153]);(
b
) new entity, or unusual variant of recognized entity, not adequately character-ized by the current American Thoracic Society/European Respiratory Society classi-fication (e.g., variant of organizing pneumonia with supervening fibrosis) (79); and(
) multiple high-resolution computed tomographyand/orpathologicpatternsthatmay be encountered in patients with idiopathic interstitial pneumonia.
734 AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE VOL 188 2013
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diseases, lung function, laboratory findings) and radiologic find-ings are essential for multidisciplinary diagnosis.The multidisciplinary approach does not lessen the impor-tance of lung biopsy in the diagnosis of IIPs; rather, it definesthe settings where biopsy is more informative than high-resolution computed tomography (HRCT) and those where bi-opsy is not needed. Also, once a pathologist has recognized a histo-logicpattern(e.g.,NSIPororganizingpneumonia[OP]),theclinicianshouldreconsiderpotentialcauses(e.g.,hypersensitivitypneumonitis[HP], collagen vascular disease [CVD], and drug exposure).
Figure 1.
 Atypicalusualintersti-tialpneumonia/idiopathic pul-monary fibrosis. Computedtomography (CT) features: (
A
)CT image of atypical usual in-terstitialpneumonia(UIP).Proneaxial CT through the lung basesin a patient with histologicallyproven UIP shows predominantlyperibronchovascular ground-glass/reticular opacities with tractionbronchiectasis. Under the ATS/ERS/JRS/ALATStatement(8),thiswould be classified as inconsistentwith UIP. Histologic features: (
)Thebiopsyshowspatchysubpleu-ral dense fibrosis with honeycombchange adjacent to preservedlung. (
) Dense scarring fibrosisis present with a small nodular fibroblasticfocus.
     4     C     /     F     P     O
Figure 2.
 Nonspecific intersti-tial pneumonia. Computed to-mography (CT) features: (
A
) Axial and (
) coronal CT recon-structions show confluent bi-lateral lower lobe ground-glass opacities with markedtraction bronchiectasis andlower lobe volume loss. Theperibronchovascular predomi-nance with subpleural sparingis well shown on the axial im-age. (
 and
 D
) Histologic fea-tures: Lung biopsy shows diffusealveolar wall thickening by uni-form fibrosis. The alveolar archi-tecture is preserved and nohoneycombing or fibroblasticfoci are seen. Interstitial inflam-mation is mild.
     4     C     /     F     P     O
 American Thoracic Society Documents 735
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