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Semin Respir Crit Care Med 2001; 22(4): 423-434
DOI: 10.1055/s-2001-17385
Copyright © 2001 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Nonspecific Interstitial Pneumonia (NSIP)

Kevin R. Flaherty1 , Fernando J. Martinez1 , William Travis2 , Joseph P. Lynch, III1
  • 1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor, Michigan
  • 2Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, D.C
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Publication History

Publication Date:
26 September 2001 (online)

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ABSTRACT

Nonspecific interstitial pneumonia (NSIP) represents one histologic subtype of idiopathic interstitial pneumonia (IIP). NSIP is typified by temporal homogeneity and less profusion of fibroblastic foci than is seen with usual interstitial pneumonia (UIP), the most common IIP. Clinically patients with NSIP present with similar symptoms (cough and dyspnea) when compared to patients with UIP. The duration of these symptoms prior to presentation is variable. The finding of fever may be more common in NSIP and clubbing may be more common in UIP; however, both findings can be seen in either UIP or NSIP. Physiological findings typically demonstrate a restrictive ventilatory defect with decreased gas transfer; little difference exists between UIP and NSIP. High resolution computed tomography (HRCT) scans are more likely to show honeycombing with UIP and a ground-glass pattern with NSIP, however, either of these findings can be seen with UIP or NSIP. The most striking differential feature between NSIP and UIP is the markedly better prognosis for patients with NSIP, a finding that cannot be explained by baseline differences in physiology or radiographic features. In this article we explore the clinical, physiological, and radiographic features of NSIP. We also review available information regarding response to therapy and prognosis.

KEYWORD

Idiopathic interstitial pneumonia - nonspecific interstitial pneumonia

REFERENCES

  • 1 Travis W D, King T E, Bateman E D. ATS/ERS International Consensus Classification of Idiopathic Interstitial Pneumonias.  Am J Respir Crit Care Med In press.
    PubMedGoogle Scholar
  • 2 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
  • 3 Ryu J H, Colby T V, Hartman T E. Idiopathic pulmonary fibrosis: current concepts.  Mayo Clin Proc . 1998;  73 1085-1101
    PubMedGoogle Scholar
  • 4 Katzenstein A LA, Fiorelli R F. Nonspecific interstitial pneumonia/fibrosis: histologic features and clinical significance.  Am J Surg Pathol . 1994;  18 136-147
    CrossrefPubMedGoogle Scholar
  • 5 Nagai S, Kitaichi M, Itoh H. Idiopathic nonspecific interstitial pneumonia/fibrosis: comparison with idiopathic pulmonary fibrosis and BOOP.  Eur Respir J . 1998;  12 1010-1019
    CrossrefPubMedGoogle Scholar
  • 6 Travis W D, Matsui K, Moss J. Idiopathic nonspecific intersititial pneumonia: prognostic signficance of cellular and fibrosing patterns.  Am J Surg Path . 2000;  24 19-33
    CrossrefPubMedGoogle Scholar
  • 7 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;  157 199-203
    CrossrefPubMedGoogle Scholar
  • 8 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;  160 899-905
    CrossrefPubMedGoogle Scholar
  • 9 Cottin V, Donsbeck A-V, Revel D. Nonspecific interstitial pneumonia: individualization of a clinicopathologic entity in a series of 12 patients.  Am J Respir Crit Care Med . 1998;  158 1286-1293
    CrossrefPubMedGoogle Scholar
  • 10 Nicholson A G, Colby T V, DuBois R M. The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis.  Am J Respir Crit Care Med . 2000;  162 2213-2217
    CrossrefPubMedGoogle Scholar
  • 11 Turner-Warwick M, Burrows B, Johnson A. Cryptogenic fibrosing alveolitis: clinical features and their influence on survival.  Thorax . 1980;  35 171-180
    CrossrefPubMedGoogle Scholar
  • 12 Watters L C, King T E, Schwarz M I. 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
  • 13 Bojko T, Notterman D A, Greenwald B M. Acute hypoxemic respiratory failure in children following bone marrow transplantation: an outcome and pathologic study.  Crit Care Med . 1995;  23 755-759
    PubMedGoogle Scholar
  • 14 Griffiths M H, Miller R F, Semple S J. Interstitial pneumonitis in patients infected with the human immunodeficiency virus.  Thorax . 1995;  50 1141-1146
    CrossrefPubMedGoogle Scholar
  • 15 Travis W D, Fox C H, Devaney K O. Lymphoid pneumonitis in 50 adult patients infected with the human immunodeficiency virus: lymphocytic interstitial pneumonitis versus nonspecific interstitial pneumonitis.  Hum Pathol . 1992;  23 529-541
    CrossrefPubMedGoogle Scholar
  • 16 Sattler F, Nichols L, Hirano L. Nonspecific interstitial pneumonitis mimicking Pneumocystis carinii pneumonia.  Am J Respir Crit Care Med . 1997;  156 912-917
    CrossrefPubMedGoogle Scholar
  • 17 Suffredini A F, Ognibene F P, Lack E E. Nonspecific interstitial pneumonitis: a common cause of pulmonary disease in the acquired immunodeficiency syndrome.  Ann Intern Med . 1987;  107 7-13
    CrossrefPubMedGoogle Scholar
  • 18 Kitaichi M. Pathologic features and the classification of interstitial pneumonia of unknown etiology.  Bull Chest Dis Res Inst Kyoto Univ . 1990;  23 1-18
    PubMedGoogle Scholar
  • 19 Tazelaar H D, Viggiano R W, Pickersgill J. 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
  • 20 Yousem S A, Colby T V, Carrington C B. Lung biopsy in rheumatoid arthritis.  Am Rev Respir Dis . 1985;  131 770-777
    PubMedGoogle Scholar
  • 21 Katzenstein A L, Myers J L. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification.  Am J Respir Crit Care Med . 1998;  157 1301-1315
    CrossrefPubMedGoogle Scholar
  • 22 Flaherty K R, Toews G B, Travis W D. Clinical significance of histopathological classification in patients with suspected idiopathic interstitial pneumonia: Results of a large, well-characterized cohort.  In review.
    PubMedGoogle Scholar
  • 23 Scott J, Johnston I, Britton J. What causes cryptogenic fibrosing alveolitis? A case control study of environmental exposure to dust.  BMJ . 1990;  301 1015-1017
    PubMedGoogle Scholar
  • 24 Iawai K, Mori T, Yamada N. Idiopathic pulmonary fibrosis: epidemiologic approaches to occupational exposure.  Am J Respir Crit Care Med . 1994;  150 670-675
    CrossrefPubMedGoogle Scholar
  • 25 Coultas D B, Zumwalt R E, Black W C. The epidemiology of interstitial lung diseases.  Am J Respir Crit Care Med . 1994;  150 967-972
    CrossrefPubMedGoogle Scholar
  • 26 Park C S, Jeon J W, Park S W. Nonspecific interstitial pneumonia/fibrosis: clinical manifestations, histologic and radiologic features.  Korean J Intern Med . 1996;  11 122-132
    CrossrefPubMedGoogle Scholar
  • 27 Fujita J, Yamadori I, Bandoh S. Clinical features of three fatal cases of nonspecific interstitial pneumonia.  Int Med . 2000;  39 407-411
    PubMedGoogle Scholar
  • 28 Nishiyama O, Kondoh Y, Taniguchi H. Serial high resolution CT findings in nonspecific interstitial pneumonia/ fibrosis.  J Comput Assist Tomogr . 2000;  24
    CrossrefPubMedGoogle Scholar
  • 29 Fujita J, Yamadori I, Suemitsu I. Clinical features of nonspecific interstitial pneumonia.  Respir Med . 1999;  93 113-118
    CrossrefPubMedGoogle Scholar
  • 30 Kim E Y, Lee K S, Chung M P. Nonspecific interstitial pneumonia with fibrosis: serial high-resolution CT findings with functional correlation.  Am J Roentgenol . 1999;  173 1734-1739
    PubMedGoogle Scholar
  • 31 Akira M, Inoue G, Yamamoto S. Nonspecific interstitial pneumonia: findings on sequential CT scans of nine patients.  Thorax . 2000;  55 854-859
    CrossrefPubMedGoogle Scholar
  • 32 Hartman T E, Swensen S J, Hansell D M. Nonspecific interstitial pneumonia: variable appearance at high-resolution chest CT.  Radiology . 2000;  217 701-705
    CrossrefPubMedGoogle Scholar
  • 33 Raghu G, Mageto Y N, Lockhart D. The accuracy of the clinical diagnosis of new-onset idiopathic pulmonary fibrosis and other interstitial lung disease: a prospective study.  Chest . 1999;  116 1168-1174
    CrossrefPubMedGoogle Scholar
  • 34 Miki H, Mio T, Hoshino Y. Production TGF-b1 and fibronectin by human lung fibroblasts: UIP vs NSIP.  Am J Resp Crit Care Med . 2000;  161 A825
    PubMedGoogle Scholar
  • 35 Jones H A, Clark R J, Rhodes C G. In vivo measurement of neutrophil activity in experimental lung inflammation.  Am J Respir Crit Care Med . 1994;  149 1635-1639
    PubMedGoogle Scholar
  • 36 Lesur O J, Mancini N M, Humbert J C. Interleukin-6, interferon-gamma, and phospholipid levels in the alveolar lining fluid of human lungs: profiles in coal workers' pneumoconiosis and idiopathic pulmonary fibrosis.  Chest . 1994;  106 407-413
    PubMedGoogle Scholar
  • 37 Miki H, Mio T, Nagai S. Fibroblast contractility: usual interstitial pneumonia and nonspecific interstitial pneumonia.  Am J Respir Crit Care Med . 2000;  162 2259-2264
    CrossrefPubMedGoogle Scholar
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