The Clinical Significance of Histopathologic Subgroups in Idiopathic Interstitial Pneumonia: Is Surgical Lung Biopsy Essential?

 

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ABSTRACT

For many years, the clinical entity of idiopathic interstitial pneumonia (IIP) has been a source of confusion for physicians. There has been much debate over the utility of subclassifying this condition histopathologically. It now appears that such classification is useful, and the most important distinction is the presence or absence of usual interstitial pneumonia (UIP). Unlike the other histopathologic subgroups, UIP has a grave prognosis and responds poorly to traditional therapies. To emphasize this clinical difference, the diagnosis of idiopathic pulmonary fibrosis (IPF), once used synonymously with IIP, is now reserved for only those patients with the histopathologic pattern of UIP. Although the gold standard for the diagnosis of IPF/UIP remains surgical lung biopsy, recent studies suggest that careful clinical and radiographic evaluation can identify IPF/UIP with a specificity of 90% or more. In the absence of a clear clinical diagnosis, we recommend pursuing surgical lung biopsy. Knowledge of the underlying histopathology will allow for more accurate prognosis, help guide therapy, and make possible the clinical investigation of novel therapeutic agents for patients with IIP.

REFERENCES

• 1 King T EJ, Costabel U, Cordier J-F. Statement on Idiopathic Pulmonary Fibrosis: Diagnosis and Treatment. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS), and the American College of Chest Physicians (ACCP) adopted by the ATS and ACCP Board of Directors and by the ERS Executive Committee.  Am J Respir Crit Care Med . 2000;  161(2 Pt 1) 646-664
  Google Scholar
• 2 du Bois M R. Diffuse lung disease: an approach to management.  BMJ . 1994;  309 175-179
  PubMedGoogle Scholar
• 3 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;  151(3 Pt 1) 909-914
  CrossrefPubMedGoogle Scholar
• 4 Schwarz M I. Approach to the understanding, diagnosis, and management of interstitial lung disease. In: Schwarz, King TE Jr, eds. Interstitial Lung Disease 3rd ed. Hamilton, Ontario, Canada: BC Decker 1998: 3-30
  Google Scholar
• 5 Michaelson J E, Aguayo S M, Roman J. Idiopathic pulmonary fibrosis: a practical approach for diagnosis and management.  Chest . 2000;  118(3) 788-794
  CrossrefPubMedGoogle Scholar
• 6 British Thoracic Society. The diagnosis, assessment and treatment of diffuse parenchymal lung disease in adults.  Thorax . 1999;  54(Suppl 1) S1-S28
  PubMedGoogle Scholar
• 7 Smith C M, Holbrook 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.  Am Rev Respir Dis . 1990;  141 A62
  PubMedGoogle Scholar
• 8 Johnston I DA, Gomm S A, Kalra S, Woodcock A A, Evans C C, Hind C RK. The management of cryptogenic fibrosing alveolitis in three regions of the United Kingdom.  Eur Respir J . 1993;  6 891-893
  PubMedGoogle Scholar
• 9 Hamman L, Rich A. Acute diffuse interstitial fibrosis of the lung.  Bull Johns Hopkins Hosp . 1944;  74 177-212
  PubMedGoogle Scholar
• 10 Liebow A A, Carrington D B. The interstitial pneumonias. In: Simon M, Potchen EJ, LeMay M, eds. Frontiers of Pulmonary Radiology New York: Grune & Stratteon 1969: 102-141
  Google Scholar
• 11 Stack B HR, Choo-Kang Y FJ, Heard B H. The prognosis of cryptogenic fibrosing alveolitis.  Thorax . 1972;  27 535-542
  CrossrefPubMedGoogle Scholar
• 12 Scadding J G, Hinson K FW. Diffuse fibrosing alveolitis (diffuse interstitial fibrosis of the lungs): corelation of histology at biopsy with prognosis.  Thorax . 1967;  22 291-304
  CrossrefPubMedGoogle Scholar
• 13 Patchefsky A S, Israel H L, Hoch W S, Gordon G. Desquamative interstitial pneumonia: relationship to interstitial fibrosis.  Thorax . 1973;  28 680-693
  PubMedGoogle Scholar
• 14 Katzenstein A L, Myers J L. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification.  Am J Respir Crit Care Med . 1998;  157(4 Pt 1) 1301-1315
  CrossrefPubMedGoogle Scholar
• 15 Travis W D, King Jr E T, Bateman E D. ATS/ERS international multidisciplinary consensus classification of idiopathic interstitial pneumonias: general principles and recommendations.  Am J Respir Crit Care Med 2001 In press.
  PubMedGoogle Scholar
• 16 Carrington C B, Gaensler E A, Coutu R E, FitzGerald M X, Gupta R G. Natural history and treated course of usual and desquamative interstitial pneumonia.  N Engl J Med . 1978;  298 801-809
  CrossrefPubMedGoogle Scholar
• 17 Bjoraker J A, Ryu J H, Edwin M K. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis.  Am J Respir Crit Care Med . 1998;  157(1) 199-203
  CrossrefPubMedGoogle Scholar
• 18 Daniil Z D, Gilchrist F C, Nicholson A G. 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;  160(3) 899-905
  CrossrefPubMedGoogle Scholar
• 19 Yousem S A, Colby T V, Gaensler E A. Respiratory bronchiolitis-associated interstitial lung disease and its relationship to desquamative interstitial pneumonia.  Mayo Clin Proc . 1989;  64 1373-1380
  CrossrefPubMedGoogle Scholar
• 20 Wells A U, Cullinan P, Hansell D M. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis.  Am J Respir Crit Care Med . 1994;  149 1583-1590
  CrossrefPubMedGoogle Scholar
• 21 Katzenstein A L, Fiorelli R F. Nonspecific interstitial pneumonia/fibrosis: histologic patterns and clinical significance.  Am J Surg Path . 1994;  18 136-147
  CrossrefPubMedGoogle Scholar
• 22 Myers J L, Veal Jr F C, Shin M S, Katzenstein A L. Respiratory bronchiolitis causing interstitial lung disease: a clinicopathologic study of six cases.  Am Rev Respir Dis . 1987;  135 880-884
  CrossrefPubMedGoogle Scholar
• 23 Nicholson A, Colby T V, du Bois M R, Hansell D M, Wells A U. 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;  162 2213-2217
  CrossrefPubMedGoogle Scholar
• 24 Nagai S, Kitaichi M, Itoh H, Nishimura K, Izumi T, Colby T V. Idiopathic nonspecific interstitial pneumonia/fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP.  European Respiratory Journal . 1998;  12 1010-1019
  CrossrefPubMedGoogle Scholar
• 25 Olsen J, Colby T V, Elliott C G. Hamman-Rich syndrome revisited.  Mayo Clin Proc . 1990;  65 1538-1548
  CrossrefPubMedGoogle Scholar
• 26 Tukiainen P, Taskinen E, Holsti P, Korhola O, Valle M. Prognosis of cryptogenic fibrosing alveolitis.  Thorax . 1983;  38 349-355
  CrossrefPubMedGoogle Scholar
• 27 Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival.  Thorax . 1980;  35 593-599
  CrossrefPubMedGoogle Scholar
• 28 Mapel D W, Samet J M, Coultas D B. Corticosteroids and the treatment of idiopathic pulmonary fibrosis: past, present, and future.  Chest . 1996;  110(4) 1058-1067
  PubMedGoogle Scholar
• 29 Gay S E, Kazerooni E A, Toews G B. Idiopathic pulmonary fibrosis: predicting response to therapy and survival.  Am J Respir Crit Care Med . 1998;  157(4 Pt 1) 1063-1072
  CrossrefPubMedGoogle Scholar
• 30 Douglas W W, Ryu J H, Swensen S J, for 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;  158 220-225
  CrossrefPubMedGoogle Scholar
• 31 Douglas W W, Ryu J H, Schroeder D R. Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival.  American Journal of Respiratory and Critical Care Medicine . 2000;  161(4 Pt 1) 1172-1178
  CrossrefPubMedGoogle Scholar
• 32 Raghu G, Depaso W J, Cain K. Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled trial.  Am Rev Respir Dis . 1991;  144 291-296
  CrossrefPubMedGoogle Scholar
• 33 Johnson M A, Snell N JC, Nunn A J, Darbyshire J H, Turner-Warwick M. Randomized controlled trial comparing prednisolone alone with cyclophosphamide and low dose prednisolone in combination in cryptogenic fibrosing alveolitis.  Thorax . 1989;  44 280-288
  CrossrefPubMedGoogle Scholar
• 34 Zisman D A, Lynch III P J, Toews G B, Kazerooni E A, Flint A, Martinez F J. Cyclophosphamide in the treatment of idiopathic pulmonary fibrosis: a prospective study in patients who failed to respond to corticosteroids.  Chest . 2000;  117(6) 1619-1626
  CrossrefPubMedGoogle Scholar
• 35 Raghu G, Johnson W C, Lockhart D, Mageto Y. Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study.  Am J Respir Crit Care Med . 1999;  159(4 Pt 1) 1061-1069
  CrossrefPubMedGoogle Scholar
• 36 Ziesche R, Hofbauer E, Wittmann K, Petkov V, Block L H. A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis [see comments].  N Engl J Med . 1999;  341(17) 1264-1269
  CrossrefPubMedGoogle Scholar
• 37 Grenier P, Chevret S, Beigelman C, Brauner M, Chastang C, Valeyre D. Chronic Diffuse Infiltrative Lung Disease: Determination of the Diagnostic Value of Clinical Data, Chest Radiography, and CT with Bayesian Analysis.  Radiology . 1994;  191 383-90
  CrossrefPubMedGoogle Scholar
• 38 Drent M, Van Nierdop M F A, Gerritsen F A, Wouters E FM, Mulder P GH. A Computer Program Using BALF-Analysis Results as a Diagnostic Tool in Interstitial Lung Diseases.  Am J Respir Crit Care Med . 1996;  153 736-41
  CrossrefPubMedGoogle Scholar
• 39 Robinson P C, Watters L C, King Jr E T, Mason R J. Idiopathic pulmonary fibrosis: abnormalities in bronchoalveolar lavage fluid phospholipids.  Am Rev Respir Dis . 1988;  137 585-591
  PubMedGoogle Scholar
• 40 Watters L C, Schwarz M I, Cherniack R M. Idiopathic pulmonary fibrosis: pretreatment bronchoalveolar lavage cellular constituents and their relationships with lung histopathology and clinical response to therapy.  Am Rev Respir Dis . 1987;  135 696-704
  PubMedGoogle Scholar
• 41 BAL Cooperative Group. Bronchoalveolar lavage constituents in healthy individuals: idiopathic pulmonary fibrosis and selected comparison groups.  Am Rev Respir Dis . 1990;  141 S169-S202
  PubMedGoogle Scholar
• 42 Haslam P L, Turton C WG, Heard B, Lukoszek J V, Salsbury A J, Turner-Warwick M. Bronchoalveolar lavage in pulmonary fibrosis: comparison of cells obtained with lung biopsy and clinical features.  Thorax . 1980;  35 9-18
  PubMedGoogle Scholar
• 43 Burt M E, Flye M W, Webber B L, Wesley R A. Prospective evaluation of aspiration needle, cutting needle, transbronchial, and open lung biopsy in patients with pulmonary infiltrates.  Ann Thorac Surg . 1981;  32(2) 146-153
  PubMedGoogle Scholar
• 44 Gilman M J, Wang K P. Transbronchial lung biopsy in Sarcoidosis.  Am Rev Respir Dis . 1980;  122 721-724
  PubMedGoogle Scholar
• 45 Wall C P, Gaensler E A, Carrington C B, Hayes J A. Comparison of transbronchial and open biopsies in chronic infiltrative lung disease.  Am Rev Respir Dis . 1981;  123 280-285
  PubMedGoogle Scholar
• 46 McLoud T C, Carrington C B, Gaensler E A. Diffuse infiltrative lung disease: a new scheme for description.  Radiology . 1983;  149 353-363
  PubMedGoogle Scholar
• 47 Mathieson J R, Mayo J R, Staples C A, Muller N. Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography.  Radiology . 1989;  171 111-116
  CrossrefPubMedGoogle Scholar
• 48 Epler G R, McLoud T C, Gaensler E A, Mikus J P, Carrington C B. Normal chest roengenograms in chronic diffuse infiltrative lung disease.  N Engl J Med . 1978;  298 934-939
  CrossrefPubMedGoogle Scholar
• 49 Liebow A A, Steer A, Billingsley J G. Desquamative interstitial pneumonia.  Am J Med . 1965;  39 369-404
  CrossrefPubMedGoogle Scholar
• 50 Winterbauer R H, Hammar S P, Hallman K O. Diffuse interstitial pneumonitis clinicopathologic correlations in 20 patients treated with prednizone/azathioprine.  Am J Med . 1978;  65 661-672
  CrossrefPubMedGoogle Scholar
• 51 Watters L C, King Jr E T, Schwarz M I, Waldron J, Stanford R E, Cherniack R M. A clinical, radiographic and physiologic scoring system for the longitudinal assessment of patients with idiopathic pulmonary fibrosis.  Am Rev Respir Dis . 1986;  133 97-103
  CrossrefPubMedGoogle Scholar
• 52 Swensen S J, Aughenbaugh G L, Brown L R. High-resolution computed tomography of the lung.  Mayo Clin Proc . 1989;  64 1284-1294
  PubMedGoogle Scholar
• 53 Felson B. A new look at pattern recognition of diffuse pulmonary disease.  AJR . 1979;  133 183-189
  PubMedGoogle Scholar
• 54 Muller N, Miller R R, Webb R W, Evans K G, Ostrow D N. Fibrosing alveolitis: CT-pathologic correlation.  Radiology . 1986;  160 585-588
  PubMedGoogle Scholar
• 55 Nishimura K, Kitaichi M, Izumi T, Nagai S, Kanaoka M, Itoh H. Usual interstitial pneumonia: histologic correlation with high-resolution CT.  Radiology . 1992;  182 337-342
  CrossrefPubMedGoogle Scholar
• 56 Orens J B, Kazerooni E A, Martinez F J. The sensitivity of high-resolution CT in detecting idiopathic pulmonary fibrosis proved by open lung biopsy: a prospective study.  Chest . 1995;  108(1) 109-115
  PubMedGoogle Scholar
• 57 Remy-Jardin M, Giraud F, Remy J, Copin M C, Gosselin B, Duhamel A. Importance of ground-glass attenuation in chronic diffuse infiltrative lung disease: pathologic-CT correlation.  Radiology . 1993;  189 693-698
  CrossrefPubMedGoogle Scholar
• 58 Nishimura K, Izumi T, Kitaichi M, Nagai S, Itoh H. The diagnostic accuracy of high-resolution computed tomography in diffuse infiltrative lung disease.  Chest . 1993;  104 1149-1155
  CrossrefPubMedGoogle Scholar
• 59 Padley S PG, Hansell D M, Flower C DR, Jennings P. Comparative accuracy of high-resolution computed tomography and chest radiography in the diagnosis of chronic diffuse infiltrative lung disease.  Clinical Radiology . 1991;  44 222-226
  CrossrefPubMedGoogle Scholar
• 60 Tung K T, Wells A U, Rubens M B, Kirk J ME, du Bois M R, Hansell D M. Accuracy of the typical computed tomographic appearances of fibrosing alveolitis.  Thorax . 1993;  48 334-338
  CrossrefPubMedGoogle Scholar
• 61 Lynch D A, Newell J D, King Jr E T, Muller N. Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?.  AJR . 1995;  165(4) 807-811
  PubMedGoogle Scholar
• 62 Hunninghake G W, Zimmerman M B, Schwartz D A, King Jr E T, Lynch III P J, Hegele R. Utility of lung biopsy for the diagnosis of idiopathic pulmonary fibrosis.  Am J Respir Crit Care Med . 2001;  164 193-196
  CrossrefPubMedGoogle Scholar
• 63 Johkoh T, Müller N L, Cartier Y. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients.  Radiology . 1999;  211(2) 555-560
  CrossrefPubMedGoogle Scholar
• 64 Raghu G, Mageto Y N, Lockhart D, Schmidt R A, Wood D E, Godwin J D. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study.  Chest . 1999;  116(5) 1168-1174
  CrossrefPubMedGoogle Scholar
• 65 Swensen S J, Aughenbaugh G L, Myers J L. Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung.  Radiology . 1997;  205(1) 229-234
  CrossrefPubMedGoogle Scholar
• 66 Gaensler E A, Carrington C B. Open biopsy for chronic diffuse infiltrative lung disease: clinical, roentgenographic, and physiological correlations in 502 patients.  Ann Thorac Surg . 1980;  30(5) 411-426
  CrossrefPubMedGoogle Scholar
• 67 Bensard D D, McIntyre Jr C R, Waring B J, Simon J S. Comparison of video thoracoscopic lung biopsy to open lung biopsy in the diagnosis of interstitial lung disease.  Chest . 1993;  103 765-770
  CrossrefPubMedGoogle Scholar
• 68 Ayed A K, Raghunathan R. Thoracoscopy versus open lung biopsy in the diagnosis of interstitial lung disease: a randomised controlled trial.  J R Coll Surg Edinb . 2000;  45(3) 159-163
  PubMedGoogle Scholar
• 69 Boutin C, Viallat J R, Cargnino P, Rey F. Thoracoscopic lung biopsy: experimental and clinical preliminary study.  Chest . 1982;  82(1) 44-48
  PubMedGoogle Scholar
• 70 Rena O, Casadio C, Leo F. Videothoracoscopic lung biopsy in the diagnosis of interstitial lung disease.  Eur J Cardiothor Surg . 1999;  16(6) 624-627
  PubMedGoogle Scholar
• 71 Zegdi R, Azorin J, Tremblay B, Destable M D, Lajos P S, Valeyre D. Videothoracoscopic lung biopsy in diffuse infiltrative lung diseases: a 5-year surgical experience.  Ann Thorac Surg . 1998;  66(4) 1170-1173
  CrossrefPubMedGoogle Scholar
• 72 Krasna M J, White C S, Aisner S C, Templeton P A, McLaughlin J S. The role of thoracoscopy in the diagnosis of interstitial lung disease.  Ann Thorac Surg . 1995;  59(2) 348-351
  CrossrefPubMedGoogle Scholar
• 73 Hartman D Z, Mylet D, Gaither J G. Comparison of thoracoscopic lung biopsy with open lung biopsy in diffuse interstitial lung disorders.  Am Rev Respir Dis . 1992;  145 A750
  PubMedGoogle Scholar
• 74 Carnochan F M, Walker W S, Cameron E W. Efficacy of video assisted thoracoscopic lung biopsy: an historical comparison with open lung biopsy.  Thorax . 1994;  49(4) 361-363
  CrossrefPubMedGoogle Scholar
• 75 Ferson P F, Landreneau R J, Dowling R D. Comparison of open versus thoracoscopic lung biopsy for diffuse infiltrative pulmonary disease.  J Thorac Cardiovasc Surg . 1993;  106(2) 194-199
  PubMedGoogle Scholar
• 76 Miller J D, Urschel J D, Cox G. A randomized, controlled trial comparing thoracoscopy and limited thoracotomy for lung biopsy in interstitial lung disease.  Ann Thorac Surg . 2000;  70 1647
  CrossrefPubMedGoogle Scholar
• 77 Travis W D, Matsui K, Moss J, Ferrans V J. Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia.  Am J Surg Path . 2000;  24(1) 19-33
  CrossrefPubMedGoogle Scholar