Download PDF
Semin Respir Crit Care Med 2001; 22(4): 399-408
DOI: 10.1055/s-2001-17383
Copyright © 2001 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Acute Interstitial Pneumonitis: Current Understanding Regarding Diagnosis, Pathogenesis, and Natural History

Jason S. Vourlekis1 2 , Kevin  K.  Brown1 2 , Marvin I. Schwarz1 2
  • 1National Jewish Medical and Research Center, Denver, Colorado
  • 2University of Colorado Health Sciences Center, Denver, Colorado
Further Information

Publication History

Publication Date:
26 September 2001 (online)

    Permissions and Reprints

ABSTRACT

Acute interstitial pneumonitis (AIP) is a fulminant disease culminating in acute respiratory failure and often death.[1] Originally described in 1935 by the pathologists Hamman and Rich, this rare syndrome is characterized by rapidly progressive pulmonary fibrosis, leading to frequent confusion with idiopathic pulmonary fibrosis.[2] In fact, the eponym Hamman-Rich syndrome became synonymous with idiopathic pulmonary fibrosis despite clear differences in clinical presentation, radiography, pathology, and survival.[3] In 1986, Katzenstein described eight patients with acute respiratory failure of unknown etiology.[4] On biopsy, organizing diffuse alveolar damage was present in all specimens. Given the idiopathic nature of the disease, Katzenstein coined the phrase acute interstitial pneumonitis to distinguish it from the fibroproliferative stage of the acute respiratory distress syndrome (ARDS), which has an identical pathology.[5] Olson et al retrospectively examined Hamman and Rich's original cases, compared them to contemporary cases of AIP, and found the two identical.[6] Since then, little progress into understanding this disease has been made. Many questions still linger regarding the epidemiology, pathogenesis, and outcome. We recently published our experience with AIP providing new information regarding natural history.[7] This review summarizes the current literature on AIP emphasizing diagnostic criteria, pathogenesis, and natural history.

KEYWORD

Acute interstitial pneumonitis - acute respiratory distress syndrome - diffuse alveolar damage - Hamman-Rich syndrome - pulmonary fibrosis

REFERENCES

  • 1 Bouros D, Nicholson A C, Polychronopoulos V, du Bois M R. Acute interstitial pneumonia.  Eur Respir J . 2000;  15 412-418
    CrossrefPubMedGoogle Scholar
  • 2 Hamman L, Rich A R. Fulminating diffuse interstitial fibrosis of the lungs.  Trans Am Clin Climat Assoc . 1935;  51 154-163
    PubMedGoogle Scholar
  • 3 Katzenstein A LA, Myers J L. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification.  Am J Respir Crit Care Med . 1998;  157 1301-1315
    CrossrefPubMedGoogle Scholar
  • 4 Katzenstein A LA, Myers J L, Mazur M T. Acute interstitial pneumonia: a clinicopathologic, ultrastructural, and cell kinetic study.  Am J Surg Pathol . 1986;  10(4) 256-267
    CrossrefPubMedGoogle Scholar
  • 5 Katzenstein A A. Acute Lung Injury Patterns: Diffuse Alveolar Damage and Bronchiolitis Obliterans-Organizing Pneumonia. Katzenstein and Askin's Surgical Pathology of Non-neoplastic Lung Disease 3rd ed. Philadelphia: WB Saunders 1997: 14-47
    Google Scholar
  • 6 Olson J, Colby T V, Elliott C G. Hamman-Rich syndrome revisited.  Mayo Clin Proc . 1990;  65 1538-1548
    CrossrefPubMedGoogle Scholar
  • 7 Vourlekis J S, Brown K K, Cool C D. Acute interstitial pneumonitis: case series and review of the literature.  Medicine . 2000;  79(6) 369-378
    PubMedGoogle Scholar
  • 8 Akira M, Hamada H, Sakatani M, Kobayashi C, Nishioka M, Yamamoto S. CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis.  Am J Roentgenol . 1997;  168 79-83
    PubMedGoogle Scholar
  • 9 Akira M. Computed tomography and pathologic findings in fulminant forms of idiopathic interstitial pneumonia.  J Thor Imaging . 1999;  14 76-84
    PubMedGoogle Scholar
  • 10 Pugin J, Verghese G, Widmer M-C, Matthay M A. The alveolar space is the site of intense inflammatory and profibrotic reactions in the early phase of acute respiratory distress syndrome.  Crit Care Med . 1999;  27 304-312
    CrossrefPubMedGoogle Scholar
  • 11 Doerschuk C M, Mizgerd J P, Kubo H, Qin L, Kumasaka T. Adhesion molecules and cellular biomechanical changes in acute lung injury.  Chest . 1999;  116(1) 37S-43S
    PubMedGoogle Scholar
  • 12 Ware L B, Matthay M A. The acute respiratory distress syndrome.  New Engl J Med . 2000;  342(18) 1334-1349
    CrossrefPubMedGoogle Scholar
  • 13 Katzenstein A A. Pathogenesis of ``fibrosis'' in interstitial pneumonia: an electron microscopic study.  Hum Pathol . 1985;  16 1015-1024
    PubMedGoogle Scholar
  • 14 Matsubara O, Tamura A, Ohdama S, Mark E J. Alveolar basement membrane breaks down in diffuse alveolar damage: an immunohistochemical study.  Pathol Intl . 1995;  45 473-482
    PubMedGoogle Scholar
  • 15 Mason R J, Shannon J M. Alveolar type II cells. In: Crystal RG, West JB, eds. The Lung: Scientific Foundations Philadelphia, PA: Lippincott-Raven Publishers; 1997
    Google Scholar
  • 16 Burkhardt A. Alveolitis and collapse in the pathogenesis of pulmonary fibrosis.  Am Rev Respir Dis . 1989;  140 513-524
    PubMedGoogle Scholar
  • 17 Guinee Jr D, Fleming M, Hayashi T. Association of p53 and WAF1 expression with apoptosis in diffuse alveolar damage.  Am J Pathol . 1996;  149(2) 531-538
    PubMedGoogle Scholar
  • 18 Guinee Jr D, Brambilla E, Fleming M. The potential role of BAX and BCL-2 expression in diffuse alveolar damage.  Am J Pathol . 1997;  151(4) 999-1007
    PubMedGoogle Scholar
  • 19 Bardales R H, Xie S S, Schaefer R F, Hsu S M. Apoptosis is a major pathway responsible for the resolution of type II pneumocytes in acute lung injury.  Am J Pathol . 1996;  149(3) 845-852
    PubMedGoogle Scholar
  • 20 Adamson A, Perkins S, Brambilla E. Proliferation, C-myc, and cyclin D11 expression in diffuse alveolar damage: potential roles in pathogenesis and implications for prognosis.  Hum Pathol . 1999;  30 1050-1057
    CrossrefPubMedGoogle Scholar
  • 21 Nash J RG, McLaughlin P J, Hoyle C, Roberts D. Immunolocalization of tumour necrosis factor a in lung tissue from patients dying with adult respiratory distress syndrome.  Histopathol . 1991;  19 395-402
    PubMedGoogle Scholar
  • 22 Pan L, Ohtani H, Yamauchi K, Nagura H. Co-expression of TNFα and IL-1β in human acute pulmonary fibrotic diseases: an immunohistochemical analysis.  Pathol Intl . 1996;  46 91-99
    PubMedGoogle Scholar
  • 23 McCormack F X. Role of pulmonary epithelium and surfactant in the pathogenesis of interstitial lung disease. In: Schwarz MI, King TE Jr, eds. Interstitial Lung Disease 3rd ed. Hamilton-London: BC Decker Inc. 1997: 165-205
    Google Scholar
  • 24 Toews G B. Cellular alterations in fibroproliferative lung disease.  Chest . 1999;  116 112S-116S
    CrossrefPubMedGoogle Scholar
  • 25 Eitzman D T, McCoy R D, Zheng X. Bleomycin-induced pulmonary fibrosis in transgenic mice that either lack or overexpress the murine plasminogen activator inhibitor-1 gene.  J Clin Invest . 1996;  97(1) 232-237
    CrossrefPubMedGoogle Scholar
  • 26 Chapman H A, Allen C L, Stone O L. Abnormalities in pathways of alveolar fibrin turnover among patients with interstitial lung disease.  Am Rev Respir Dis . 1986;  133 437-443
    PubMedGoogle Scholar
  • 27 Hasday J D, Bachwich P R, Lynch III P J, Sitrin R G. Procoagulant and plasminogen activator activities of bronchoalveolar fluid in patients with pulmonary sarcoidosis.  Exp Lung Res . 1988;  14 261-278
    PubMedGoogle Scholar
  • 28 Gross T J, Simon R H, Kelly C J, Sitrin R G. Rat alveolar epithelial cells concomitantly express plasminogen activator inhibitor-1 and urokinase.  Am J Physiol . 1991;  260(4) L286-L295
    PubMedGoogle Scholar
  • 29 Idell S, Gonzalez K, Bradford H. Procoagulant activity in bronchoalveolar lavage in the adult respiratory distress syndrome: contribution of tissue factor associated with factor VII.  Am Rev Respir Dis . 1987;  136 1466-1474
    PubMedGoogle Scholar
  • 30 Idell S, James K K, Levin E G. Local abnormalities in coagulation and fibrinolytic pathways predispose to alveolar fibrin deposition in the adult respiratory distress syndrome.  J Clin Invest . 1989;  84 695-705
    CrossrefPubMedGoogle Scholar
  • 31 Liu J, Brass D M, Hoyle G W, Brody A R. TNF-α receptor knockout mice are protected from the fibroproliferative effects of inhaled asbestos fibers.  Am J Pathol . 1998;  153(6) 1839-1847
    PubMedGoogle Scholar
  • 32 Brass D M, Hoyle G W, Poovey H G, Liu J, Brody A R. Reduced tumor necrosis factor-a and transforming growth factor-β1 expression in the lungs of inbred mice that fail to develop fibroproliferative lesions consequent to asbestos exposure.  Am J Pathol . 1999;  154(3) 853-862
    PubMedGoogle Scholar
  • 33 Sime P J, Marr R A, Gauldie D. Transfer of tumor necrosis factor-a to rat lung induces severe pulmonary inflammation and patchy interstitial fibrogenesis with induction of transforming growth factor-b1 and myofibroblasts.  Am J Pathol . 1998;  153(3) 825-832
    CrossrefPubMedGoogle Scholar
  • 34 Kahari V, Chen Y Q, Su M W, Ramirez F, Uitto J. Tumor necrosis factor-a and interferon-g suppress the activation of human type I collagen gene expression by transforming growth factor-β1: evidence for two distinct mechanisms of inhibition at the transcriptional and posttranscriptional levels.  J Clin Invest . 1990;  86 1489-1495
    PubMedGoogle Scholar
  • 35 Strieter R M, Keane M P, Standiford T J, Kunkel S L. Cytokine Biology and the Pathogenesis of Interstitial Lung Disease. In: Schwarz MI, King TE Jr, eds. Interstitial Lung Disease 3rd ed. Hamilton-London: BC Decker Inc. 1997: 181-205
    Google Scholar
  • 36 Desmouliere A, Geinoz A, Gabbiani F, Gabbiani G. Transforming growth factor-β1 induces a-smooth muscle actin expression in granulation tissue myofibroblasts and in quiescent and growing cultured fibroblasts.  J Cell Biol . 1993;  122 103-111
    CrossrefPubMedGoogle Scholar
  • 37 Chan E D, Worthen G S, Augustin A, Lapadat R, Riches D WH. Inflammation in the pathogenesis of interstitial lung diseases. In: Schwarz MI, King TE Jr, eds. Interstitial Lung Disease 3rd ed. Hamilton-London: BC Decker Inc. 1997: 135-164
    Google Scholar
  • 38 Thornton S C, Por S B, Wlash B J, Penny R, Breit S N. Interaction of immune and connective tissue cells: the effect of lymphokines and monokines on fibroblast growth.  J Leukoc Biol . 1990;  47 312-320
    PubMedGoogle Scholar
  • 39 Snyder L S, Hertz M I, Peterson M S. Acute lung injury: pathogenesis of intraalveolar fibrosis.  J Clin Invest . 1991;  88 663-673
    PubMedGoogle Scholar
  • 40 Pache J, Christakos P G, Gannon D E, Mitchell J J, Low R B, Leslie K O. Myofibroblasts in diffuse alveolar damage of the lung.  Mod Pathol . 1998;  11(11) 1064-1070
    PubMedGoogle Scholar
  • 41 Hayashi T, Stetler-Stevenson W G, Fleming M. Immunohistochemical study of metalloproteinases and their tissue inhibitors in the lungs of patients with diffuse alveolar damage and idiopathic pulmonary fibrosis.  Am J Pathol . 1996;  149(4) 1241-1256
    PubMedGoogle Scholar
  • 42 Raghu G, Striker L J, Hudson L D, Striker G E. Extracellular matrix in normal and fibrotic human lungs.  Am Rev Respir Dis . 1985;  131 281-289
    PubMedGoogle Scholar
  • 43 Kuhn III C, Boldt J, King Jr E T, Crouch E, Vartio T, McDonald J A. An immunohistochemical study of architectural remodeling and connective tissue synthesis in pulmonary fibrosis.  Am Rev Respir Dis . 1989;  140 1693-1703
    PubMedGoogle Scholar
  • 44 Specks U, Nerlich A, Colby T V, Wiest I, Timpl R. Increased expression of type VI collagen in lung fibrosis.  Am J Respir Crit Care Med . 1995;  151 1956-1964
    PubMedGoogle Scholar
  • 45 McDonald J A, Kelley D G, Broekelmann T S. Role of fibronectin in collagen deposition: fab to the gelatin-binding domain of fibronectin inhibits both fibronectin and collagen organization in fibroblast extracellular matrix.  J Cell Biol . 1982;  92 485-492
    CrossrefPubMedGoogle Scholar
  • 46 Marinelli W A, Henke C A, Harmon K R, Hertz M I, Bitterman P B. Mechanism of alveolar fibrosis after acute lung injury.  Clin Chest Med . 1990;  11(4) 657-672
    PubMedGoogle Scholar
  • 47 Razzaque M S, Nazneen A, Taguchi T. Immunolocalization of collagen and collagen-binding heat shock protein 47 in fibrotic lung diseases.  Mod Pathol . 1998;  11(12) 1183-1188
    PubMedGoogle Scholar
  • 48 Zapol W M, Trelstad R L, Coffey J W, Tsai I, Salvador R A. Pulmonary fibrosis in severe acute respiratory failure.  Am Rev Respir Dis . 1979;  119 547-554
    PubMedGoogle Scholar
  • 49 Meduri G U, Eltorky M, Winer-Muram H T. The Fibroproliferative phase of late adult respiratory distress syndrome.  Seminars Respir Infections . 1995;  10(3) 154-175
    PubMedGoogle Scholar
  • 50 Selman M, King Jr E T, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy.  Ann Intern Med . 2001;  134 136-151
    CrossrefPubMedGoogle Scholar
  • 51 Herrick S E, Sloan P, McGurk M, Freak L, McCollum C N, Ferguson M WJ. Sequential changes in histologic pattern and extracellular matrix deposition during the healing of chronic venous ulcers.  Am J Pathol . 1992;  141(5) 1085-1095
    PubMedGoogle Scholar
  • 52 Desmouliere A, Redard M, Darby I, Gabbiani G. Apoptosis mediates the decrease in cellularity during the transition between granulation tissue and scar.  Am J Pathol . 1995;  146(1) 56-66
    PubMedGoogle Scholar
  • 53 Collins J F, Smith J D, Coalson J J, Johanson Jr G W. Variability in lung collagen amounts after prolonged support of acute respiratory failure.  Chest . 1984;  85(5) 641-646
    PubMedGoogle Scholar
  • 54 Raghu G, Mageto Y N. Genetic predisposition of interstitial lung disease. In: Schwarz MI, King TE Jr, eds. Interstitial Lung Disease 3rd ed. Hamilton-London: BC Decker Inc. 1998: 119-134
    Google Scholar
  • 55 Awad M R, El-Gamel A, Hasleton P, Turner D M, Sinnott P J, Hutchinson I V. Genotypic variation in the transforming growth factor-β1 gene.  Transplantation . 1998;  66 1014-1020
    CrossrefPubMedGoogle Scholar
  • 56 Olsen N J, Wortmann R L. Inflammatory and metabolic diseases of muscle. In: Klippel JH, ed. Primer on the Rheumatic Diseases 11th ed. Atlanta: The Arthritis Foundation 1997: 276-282
    Google Scholar
  • 57 Criswell L A, Amos C I. Update on genetic risk factors for systemic lupus erythematosus and rheumatoid arthritis.  Curr Opin Rheum . 2000;  12(2) 85-90
    PubMedGoogle Scholar
  • 58 Yazdy A M, Tomashefski J FJ, Yagan R, Kleinerman J. Regional alveolar damage: a localized counterpart of diffuse alveolar damage.  Am J Clin Pathol . 1989;  92 10-15
    PubMedGoogle Scholar
  • 59 Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbations in idiopathic pulmonary fibrosis: analysis of clinical and pathologic findings in three cases.  Chest . 1993;  103 1808-1812
    CrossrefPubMedGoogle Scholar
  • 60 Schwarz M I. The acute (noninfectious) interstitial lung diseases.  Comprehensive Therapy . 1996;  22(10) 622-630
    PubMedGoogle Scholar
  • 61 Primack S L, Hartman T E, Ikezoe J, Akira M, Sakatani M, Muller N L. Acute interstitial pneumonia: radiographic and CT findings in nine patients.  Radiology . 1993;  188 817-820
    CrossrefPubMedGoogle Scholar
  • 62 Johkoh T, Muller N L, Taniguchi H. Acute interstitial pneumonia: thin-section CT findings in 36 patients.  Radiology . 1999;  211 859-863
    CrossrefPubMedGoogle Scholar
  • 63 Ichikado K, Johkoh T, Ikezoe J. Acute interstitial pneumonia: high-resolution CT findings correlated with pathology.  Am J Roentgenol . 1997;  168 333-338
    PubMedGoogle Scholar
  • 64 Robinson D S, Geddes D M, Hansell D M. Partial resolution of acute interstitial pneumonia in native lung after single lung transplantation.  Thorax . 1996;  51 1158-1159
    PubMedGoogle Scholar
  • 65 Hamman L, Rich A R. Acute diffuse interstitial fibrosis of the lungs.  Bull Hopkins Hosp . 1944;  74 177-212
    PubMedGoogle Scholar
  • 66 Warner D O, Warner M A, Divertie M B. Open lung biopsy in patients with diffuse pulmonary infiltrates and acute respiratory failure.  Am Rev Respir Dis . 1988;  137 90-94
    PubMedGoogle Scholar
  • 67 Matthay R A, Schwarz M I, Petty T L. Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases of acute lupus pneumonitis.  Medicine . 1974;  54(5) 397-409
    PubMedGoogle Scholar
  • 68 Pratt D S, Schwarz M I, May J J, Dreisin R B. Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis.  Thorax . 1979;  34 587-593
    PubMedGoogle Scholar
  • 69 Tazelaar H D, Viggiano R W, Pickersgill J, Colby T V. Interstitial lung disease in polymyositis and dermatomyositis: clinical features and prognosis as correlated with histologic findings.  Am Rev Respir Dis . 1990;  141 727-733
    CrossrefPubMedGoogle Scholar
  • 70 Kreidstein S H, Lytwyn A, Keystone E C. Takayasu arteritis with acute interstitial pneumonia and coronary vasculitis: expanding the spectrum.  Arthr Rheum . 1993;  36(8) 1175-1178
    PubMedGoogle Scholar
  • 71 Akikusa B, Kondo Y, Irabu N, Yamamoto S, Saiki S. Six cases of microscopic polyarteritis exhibiting acute interstitial pneumonia.  Pathol Intl . 1995;  45 580-588
    CrossrefPubMedGoogle Scholar
  • 72 Muir T E, Tazelaar H D, Colby T V, Myers J L. Organizing diffuse alveolar damage associated with progressive systemic sclerosis.  Mayo Clin Proc . 1997;  72 639-642
    CrossrefPubMedGoogle Scholar
  • 73 Meduri G U, Headley A S, Golden E. Effect of prolonged methylprednisolone therapy in unresolving acute respiratory distress syndrome: a randomized controlled trial.  JAMA . 1998;  280(2) 159-165
    CrossrefPubMedGoogle Scholar
  • 74 Behrendt C E. Acute respiratory failure in the United States: incidence and 31-day survival.  Chest . 2000;  118 1100-1105
    CrossrefPubMedGoogle Scholar
  • 75 Milberg J A, Davis D R, Steinberg K P, Hudson L D. Improved survival of patients with acute respiratory distress syndrome (ARDS): 1983-1993.  JAMA . 1995;  273(4) 306-309
    CrossrefPubMedGoogle Scholar
  • 76 Martin C, Papazian L, Payan M, Saux P, Gouin F. Pulmonary fibrosis correlates with outcome in adult respiratory distress syndrome: a study in mechanically ventilated patients.  Chest . 1995;  107 196-200
    CrossrefPubMedGoogle Scholar
  • 77 Brower R G, Matthay M A, Morris A H, Schoenfeld D, Thompson B T, Wheeler A. Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome.  New Engl J Med . 2000;  342(18) 1301-1308
    CrossrefPubMedGoogle Scholar
  • 78 Gunning K, Rowan K. ABC of intensive care: outcome data and scoring systems.  BMJ . 1999;  319(7204) 241-244
    PubMedGoogle Scholar
  • 79 Davidson T A, Rubenfeld G D, Caldwell E S, Hudson L D, Steinberg K. The effect of acute respiratory distress syndrome on long-term survival.  Am J Respir Crit Care Med . 1999;  160 1838-1842
    PubMedGoogle Scholar
  • 80 McHugh L G, Milberg J A, Whitcomb M E, Schoene R B, Maunder R J, Hudson L J. Recovery of function in survivors of the acute respiratory distress syndrome.  Am J Respir Crit Care Med . 1994;  150 90-94
    CrossrefPubMedGoogle Scholar
      >