Demystifying Idiopathic Interstitial Pneumonia | Pulmonary Medicine | JAMA Internal Medicine | JAMA Network
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1.
Osler  W The Principles and Practice of Medicine.  New York, NY D Appleton & Co1892;
2.
Hamman  LRich  A Acute diffuse interstitial fibrosis of the lung.  Bull Johns Hopkins Hosp. 1944;74177- 212Google Scholar
3.
Liebow  AACarrington  CB The interstitial pneumonias. Simon  MPotchen  EJLeMay  Meds. Frontiers of Pulmonary Radiology. New York, NY Grune & Stratton Inc1969;102- 141Google Scholar
4.
Katzenstein  ALMyers  JL Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification.  Am J Respir Crit Care Med. 1998;157 ((pt 1)) 1301- 1315Google ScholarCrossref
5.
Not Available, American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of idiopathic interstitial pneumonias: general principles and recommendations.  Am J Respir Crit Care Med. 2002;165277- 304Google ScholarCrossref
6.
Vourlekis  JSBrown  KKCool  CD  et al.  Acute interstitial pneumonitis: case series and review of the literature.  Medicine (Baltimore). 2000;79369- 378Google ScholarCrossref
7.
Bjoraker  JARyu  JHEdwin  MK  et al.  Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis.  Am J Respir Crit Care Med. 1998;157199- 203Google ScholarCrossref
8.
Nagai  SKitaichi  MItoh  HNishimura  KIzumi  TColby  TV Idiopathic nonspecific interstitial pneumonia/fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP.  Eur Respir J. 1998;121010- 1019Google ScholarCrossref
9.
Nicholson  AColby  TVdu Bois  RMHansell  DMWells  AU The prognostic significance of the histologic pattern of interstitial pneumonia in patients with the clinical entity of cryptogenic fibrosing alveolitis.  Am J Respir Crit Care Med. 2000;1622213- 2217Google ScholarCrossref
10.
Travis  WDMatsui  KMoss  JFerrans  VJ Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia.  Am J Surg Pathol. 2000;2419- 33Google ScholarCrossref
11.
Collard  HKing  T  Jr The clinical significance of histopathologic subgroups in idiopathic interstitial pneumonia: is surgical lung biopsy essential?  Semin Respir Crit Care Med. 2001;22347- 356Google ScholarCrossref
12.
Katzenstein  ALFiorelli  RF Nonspecific interstitial pneumonia/fibrosis: histologic patterns and clinical significance.  Am J Surg Pathol. 1994;18136- 147Google ScholarCrossref
13.
Myers  JLVeal  CF  JrShin  MSKatzenstein  AL Respiratory bronchiolitis causing interstitial lung disease: a clinicopathologic study of six cases.  Am Rev Respir Dis. 1987;135880- 884Google Scholar
14.
Yousem  SAColby  TVGaensler  EA Respiratory bronchiolitis–associated interstitial lung disease and its relationship to desquamative interstitial pneumonia.  Mayo Clin Proc. 1989;641373- 1380Google ScholarCrossref
15.
Mapel  DWSamet  JMCoultas  DB Corticosteroids and the treatment of idiopathic pulmonary fibrosis: past, present, and future.  Chest. 1996;1101058- 1067Google ScholarCrossref
16.
Tukiainen  PTaskinen  EHolsti  PKorhola  OValle  M Prognosis of cryptogenic fibrosing alveolitis.  Thorax. 1983;38349- 355Google ScholarCrossref
17.
Turner-Warwick  MBurrows  BJohnson  A Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival.  Thorax. 1980;35593- 599Google ScholarCrossref
18.
Stack  BHRChoo-Kang  YFJHeard  BH The prognosis of cryptogenic fibrosing alveolitis.  Thorax. 1972;27535- 542Google ScholarCrossref
19.
Gay  SEKazerooni  EAToews  GB  et al.  Idiopathic pulmonary fibrosis: predicting response to therapy and survival.  Am J Respir Crit Care Med. 1998;1571063- 1072Google ScholarCrossref
20.
Flaherty  KRToews  GBLynch  DA  et al.  Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival.  Am J Med. 2001;110278- 282Google ScholarCrossref
21.
Carrington  CBGaensler  EACoutu  REFitzGerald  MXGupta  RG Natural history and treated course of usual and desquamative interstitial pneumonia.  N Engl J Med. 1978;298801- 809Google ScholarCrossref
22.
Douglas  WWRyu  JHSwensen  SJ  et al. and Members of the Lung Study Group, Colchicine versus prednisone in the treatment of idiopathic pulmonary fibrosis: a randomized prospective study.  Am J Respir Crit Care Med. 1998;158220- 225Google ScholarCrossref
23.
Daniil  ZDGilchrist  FCNicholson  AG  et al.  A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis.  Am J Respir Crit Care Med. 1999;160899- 905Google ScholarCrossref
24.
Olson  JColby  TVElliott  CG Hamman-Rich syndrome revisited.  Mayo Clin Proc. 1990;651538- 1548Google ScholarCrossref
25.
Raghu  GDepaso  WJCain  K  et al.  Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled trial.  Am Rev Respir Dis. 1991;144291- 296Google ScholarCrossref
26.
Johnson  MASnell  NJCNunn  AJDarbyshire  JHTurner-Warwick  M Randomized controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis.  Thorax. 1989;44280- 288Google ScholarCrossref
27.
Zisman  DALynch III  JPToews  GBKazerooni  EAFlint  AMartinez  FJ Cyclophosphamide in the treatment of idiopathic pulmonary fibrosis: a prospective study in patients who failed to respond to corticosteroids.  Chest. 2000;1171619- 1626Google ScholarCrossref
28.
Douglas  WWRyu  JHSchroeder  DR Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival.  Am J Respir Crit Care Med. 2000;1611172- 1178Google ScholarCrossref
29.
Ziesche  RHofbauer  EWittmann  KPetkov  VBlock  LH A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis.  N Engl J Med. 1999;3411264- 1269Google ScholarCrossref
30.
Kolb  MKirschner  JRiedel  WWirtz  HSchmidt  M Cyclophosphamide pulse therapy in idiopathic pulmonary fibrosis.  Eur Respir J. 1998;121409- 1414Google ScholarCrossref
31.
Smith  CMHolbrook  T Utilization of the transbronchial biopsy (TBB) and open lung biopsy for tissue to establish the diagnosis of idiopathic pulmonary fibrosis: survey of the California Thoracic Society membership [abstract].  Am Rev Respir Dis. 1990;141A62Google ScholarCrossref
32.
Johnston  IDAGomm  SAKalra  SWoodcock  AAEvans  CCHind  CRK The management of cryptogenic fibrosing alveolitis in three regions of the United Kingdom.  Eur Respir J. 1993;6891- 893Google Scholar
33.
The American Society for Transplant Physicians (ASTP)/American Thoracic Society (ATS)/European Respiratory Society (ERS)/International Society for Heart and Lung Transplantation (ISHLT)., International guidelines for the selection of lung transplant candidates.  Am J Respir Crit Care Med. 1998;158335- 339Google ScholarCrossref
34.
Meester  JDSmits  JMPersijn  GGHaverich  A Lung transplant waiting list: differential outcome of type of end-stage lung disease, one year after registration.  J Heart Lung Transplant. 1999;18563- 571Google ScholarCrossref
35.
Mogulkoc  NBrutsche  MHBishop  PWGreaves  SMHorrocks  AWEgan  JJ Pulmonary function in idiopathic pulmonary fibrosis and referral for lung transplantation.  Am J Respir Crit Care Med. 2001;164103- 108Google ScholarCrossref
36.
Meyers  BFLynch  JPTrulock  EPGuthrie  TCooper  JDPatterson  GA Single versus bilateral lung transplantation for idiopathic pulmonary fibrosis: a ten-year institutional experience.  J Thorac Cardiovasc Surg. 2000;12099- 107Google ScholarCrossref
37.
Grossman  RFFrost  AZamel  N  et al.  Results of single-lung transplantation for bilateral pulmonary fibrosis.  N Engl J Med. 1990;322727- 733Google ScholarCrossref
38.
Hosenpud  JDBennett  LEKeck  BM  et al.  Effect of diagnosis on survival benefit of lung transplantation for end-stage lung disease.  Lancet. 1998;35124- 27Google ScholarCrossref
39.
Wells  AUCullinan  PHansell  DM  et al.  Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis.  Am J Respir Crit Care Med. 1994;1491583- 1590Google ScholarCrossref
40.
King  TE  JrSchwarz  MIBrown  K  et al.  Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality.  Am J Respir Crit Care Med. 2001;1641025- 1032Google ScholarCrossref
41.
Selman  MKing  TE  JrPardo  A Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy.  Ann Intern Med. 2001;134136- 151Google ScholarCrossref
42.
American Thoracic Society, Idiopathic pulmonary fibrosis: diagnosis and treatment: joint statement of the American Thoracic Society (ATS) and the European Respiratory Society (ERS).  Am J Respir Crit Care Med. 2000;161646- 664Google ScholarCrossref
43.
Michaelson  JEAguayo  SMRoman  J Idiopathic pulmonary fibrosis: a practical approach for diagnosis and management.  Chest. 2000;118788- 794Google ScholarCrossref
44.
British Thoracic Society, The diagnosis, assessment and treatment of diffuse parenchymal lung disease in adults.  Thorax. 1999;54(suppl 1)S1- S28Google Scholar
45.
Raghu  G Interstitial lung disease: a diagnostic approach: are CT scan and lung biopsy indicated in every patient?  Am J Respir Crit Care Med. 1995;151909- 914Google ScholarCrossref
46.
du Bois  RM Diffuse lung disease: an approach to management.  BMJ. 1994;309175- 179Google ScholarCrossref
47.
Schwarz  MI Approach to the understanding, diagnosis, and management of interstitial lung disease. Schwarz  MIKing  TE  Jreds. Interstitial Lung Disease. 3rd ed. Burlington, Ontario BC Decker Inc1998;3- 30Google Scholar
48.
Grenier  PChevret  SBeigelman  CBrauner  MChastang  CValeyre  D Chronic diffuse infiltrative lung disease: determination of the diagnostic value of clinical data, chest radiography, and CT with Bayesian analysis.  Radiology. 1994;191383- 390Google Scholar
49.
Drent  MVan Nierdop  AMFGerritsen  FAWouters  EFMMulder  PGH A computer program using BALF-analysis results as a diagnostic tool in interstitial lung diseases.  Am J Respir Crit Care Med. 1996;153736- 741Google ScholarCrossref
50.
Robinson  PCWatters  LCKing  TE  JrMason  RJ Idiopathic pulmonary fibrosis: abnormalities in bronchoalveolar lavage fluid phospholipids.  Am Rev Respir Dis. 1988;137585- 591Google ScholarCrossref
51.
Watters  LCSchwarz  MICherniack  RM  et al.  Idiopathic pulmonary fibrosis: pretreatment bronchoalveolar lavage cellular constituents and their relationships with lung histopathology and clinical response to therapy.  Am Rev Respir Dis. 1987;135696- 704Google Scholar
52.
Bronchoalveolar lavage constituents in healthy individuals pulmonary fibrosis and selected comparison groups idiopathic, Am Rev Respir Dis. 1990;141(suppl)S169- S196
53.
Haslam  PLTurton  CWGHeard  BLukoszek  JVSalsbury  AJTurner-Warwick  M Bronchoalveolar lavage in pulmonary fibrosis: comparison of cells obtained with lung biopsy and clinical features.  Thorax. 1980;359- 18Google ScholarCrossref
54.
Burt  MEFlye  MWWebber  BLWesley  RA Prospective evaluation of aspiration needle, cutting needle, transbronchial, and open lung biopsy in patients with pulmonary infiltrates.  Ann Thorac Surg. 1981;32146- 153Google ScholarCrossref
55.
Gilman  MJWang  KP Transbronchial lung biopsy in sarcoidosis.  Am Rev Respir Dis. 1980;122721- 724Google Scholar
56.
Wall  CPGaensler  EACarrington  CBHayes  JA Comparison of transbronchial and open biopsies in chronic infiltrative lung disease.  Am Rev Respir Dis. 1981;123280- 285Google Scholar
57.
McLoud  TCCarrington  CBGaensler  EA Diffuse infiltrative lung disease: a new scheme for description.  Radiology. 1983;149353- 363Google Scholar
58.
Mathieson  JRMayo  JRStaples  CAMuller  N Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography.  Radiology. 1989;171111- 116Google Scholar
59.
Epler  GRMcLoud  TCGaensler  EAMikus  JPCarrington  CB Normal chest roentgenograms in chronic diffuse infiltrative lung disease.  N Engl J Med. 1978;298934- 939Google ScholarCrossref
60.
Liebow  AASteer  ABillingsley  JG Desquamative interstitial pneumonia.  Am J Med. 1965;39369- 404Google ScholarCrossref
61.
Winterbauer  RHHammar  SPHallman  KO  et al.  Diffuse interstitial pneumonitis clinicopathologic correlations in 20 patients treated with prednisone/azathioprine.  Am J Med. 1978;65661- 672Google ScholarCrossref
62.
Watters  LCKing  TE  JrSchwarz  MIWaldron  JStanford  RECherniack  RM A clinical, radiographic and physiologic scoring system for the longitudinal assessment of patients with idiopathic pulmonary fibrosis.  Am Rev Respir Dis. 1986;13397- 103Google Scholar
63.
Swensen  SJAughenbaugh  GLBrown  LR High-resolution computed tomography of the lung.  Mayo Clin Proc. 1989;641284- 1294Google ScholarCrossref
64.
Felson  B A new look at pattern recognition of diffuse pulmonary disease.  AJR Am J Roentgenol. 1979;133183- 189Google ScholarCrossref
65.
Muller  NMiller  RRWebb  RWEvans  KGOstrow  DN Fibrosing alveolitis: CT-pathologic correlation.  Radiology. 1986;160585- 588Google Scholar
66.
Nishimura  KKitaichi  MIzumi  TNagai  SKanaoka  MItoh  H Usual interstitial pneumonia: histologic correlation with high-resolution CT.  Radiology. 1992;182337- 342Google Scholar
67.
Orens  JBKazerooni  EAMartinez  FJ  et al.  The sensitivity of high-resolution CT in detecting idiopathic pulmonary fibrosis proved by open lung biopsy: a prospective study.  Chest. 1995;108109- 115Google ScholarCrossref
68.
Remy-Jardin  MGiraud  FRemy  JCopin  MCGosselin  BDuhamel  A Importance of ground glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation.  Radiology. 1993;189693- 698Google Scholar
69.
Nishimura  KIzumi  TKitaichi  MNagai  SItoh  H The diagnostic accuracy of high-resolution computed tomography in diffuse infiltrative lung disease.  Chest. 1993;1041149- 1155Google ScholarCrossref
70.
Padley  SPGHansell  DMFlower  CDRJennings  P Comparative accuracy of high-resolution computed tomography and chest radiography in the diagnosis of chronic diffuse infiltrative lung disease.  Clin Radiol. 1991;44222- 226Google ScholarCrossref
71.
Tung  KTWells  AURubens  MBKirk  JMEdu Bois  RMHansell  DM Accuracy of the typical computed tomographic appearances of fibrosing alveolitis.  Thorax. 1993;48334- 338Google ScholarCrossref
72.
Lynch  DANewell  JDKing  TE  JrMuller  N Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?  AJR Am J Roentgenol. 1995;165807- 811Google ScholarCrossref
73.
Hunninghake  GWZimmerman  MBSchwartz  DA  et al.  Utility of a lung biopsy for the diagnosis of idiopathic pulmonary fibrosis.  Am J Respir Crit Care Med. 2001;164193- 196Google ScholarCrossref
74.
Johkoh  TMüller  NLCartier  Y  et al.  Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients.  Radiology. 1999;211555- 560Google ScholarCrossref
75.
Raghu  GMageto  YNLockhart  DSchmidt  RAWood  DEGodwin  JD The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study.  Chest. 1999;1161168- 1174Google ScholarCrossref
76.
Swensen  SJAughenbaugh  GLMyers  JL Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung.  Radiology. 1997;205229- 234Google Scholar
77.
Gaensler  EACarrington  CB Open biopsy for chronic diffuse infiltrative lung disease: clinical, roentgenographic, and physiological correlations in 502 patients.  Ann Thorac Surg. 1980;30411- 426Google ScholarCrossref
78.
Bensard  DDMcIntyre  RC  JrWaring  BJSimon  JS Comparison of video thoracoscopic lung biopsy to open lung biopsy in the diagnosis of interstitial lung disease.  Chest. 1993;103765- 770Google ScholarCrossref
79.
Ayed  AKRaghunathan  R Thoracoscopy versus open lung biopsy in the diagnosis of interstitial lung disease: a randomised controlled trial.  J R Coll Surg Edinb. 2000;45159- 163Google Scholar
80.
Boutin  CViallat  JRCargnino  PRey  F Thoracoscopic lung biopsy: experimental and clinical preliminary study.  Chest. 1982;8244- 48Google ScholarCrossref
81.
Rena  OCasadio  CLeo  F  et al.  Videothoracoscopic lung biopsy in the diagnosis of interstitial lung disease.  Eur J Cardiothorac Surg. 1999;16624- 627Google ScholarCrossref
82.
Zegdi  RAzorin  JTremblay  BDestable  MDLajos  PSValeyre  D Videothoracoscopic lung biopsy in diffuse infiltrative lung diseases: a 5-year surgical experience.  Ann Thorac Surg. 1998;661170- 1173Google ScholarCrossref
83.
Krasna  MJWhite  CSAisner  SCTempleton  PAMcLaughlin  JS The role of thoracoscopy in the diagnosis of interstitial lung disease.  Ann Thorac Surg. 1995;59348- 351Google ScholarCrossref
84.
Hartman  DZMylet  DGaither  JG  et al.  Comparison of thoracoscopic lung biopsy with open lung biopsy in diffuse interstitial lung disorders [abstract].  Am Rev Respir Dis. 1992;145A750Google ScholarCrossref
85.
Carnochan  FMWalker  WSCameron  EW Efficacy of video assisted thoracoscopic lung biopsy: an historical comparison with open lung biopsy.  Thorax. 1994;49361- 363Google ScholarCrossref
86.
Ferson  PFLandreneau  RJDowling  RD  et al.  Comparison of open versus thoracoscopic lung biopsy for diffuse infiltrative pulmonary disease.  J Thorac Cardiovasc Surg. 1993;106194- 199Google Scholar
87.
Miller  JDUrschel  JDCox  G  et al.  A randomized, controlled trial comparing thoracoscopy and limited thoracotomy for lung biopsy in interstitial lung disease.  Ann Thorac Surg. 2000;701647- 1650Google ScholarCrossref
Review
January 13, 2003

Demystifying Idiopathic Interstitial Pneumonia

Author Affiliations

From the Departments of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver (Dr Collard), and San Francisco General Hospital, University of California–San Francisco (Dr King). Dr King was the chairman of the advisory committee of a multicenter study of the efficacy of interferon beta-1a (Avonex) in the treatment of idiopathic pulmonary fibrosis, sponsored by Biogen Inc, Cambridge, Mass, and is a member of the Scientific Advisory Committee for InterMune Protocol GIPF-001 sponsored by InterMune Pharmaceuticals Inc, Burlington, Calif, for which he receives consulting fees.

Arch Intern Med. 2003;163(1):17-29. doi:10.1001/archinte.163.1.17
Abstract

Careful histopathological evaluation has shown the traditionally clinical diagnosis of idiopathic interstitial pneumonia to be more heterogeneous than once thought. Its subclassification, based on clinicopathological criteria, has important therapeutic and prognostic implications. The most important distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of idiopathic pulmonary fibrosis suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. Although the clinical and radiographic diagnosis of idiopathic pulmonary fibrosis can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathological pattern. A structured, clinicopathological approach to the diagnosis of idiopathic interstitial pneumonia, with particular attention to the identification of idiopathic pulmonary fibrosis, ensures proper therapy, enhances prognosis, and allows for further investigation of therapies aimed at the distinct pathophysiology.

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