Clinical study
Clinical deterioration in patients with idiopathic pulmonary fibrosis: Causes and assessment

https://doi.org/10.1016/0002-9343(90)90495-YGet rights and content

Abstract

Patients with idiopathic pulmonary fibrosis (IPF) inevitably experience declines in functional status that are most frequently due to progressive pulmonary fibrosis. However, the cause of the clinical deterioration is often uncertain, and disease progression is difficult to distinguish from disease-associated complications or adverse effects of therapy. In studies of the clinical course of IPF, mortality is most frequently due to respiratory failure (38.7%); other causes of death include heart failure (14.4%), bronchogenic carcinoma (10.4%), ischemic heart disease (9.5%), infection (6.5%), and pulmonary embolism (3.4%). Other, usually nonfatal, disease-associated complications include pneumothorax, corticosteroid-induced metabolic side effects and myopathy, and therapy-related immunosuppression. In evaluating clinical deterioration in patients with IPF, disease-associated complications and adverse effects of therapy should be distinguished from progressive pulmonary fibrosis. The cause of clinical deterioration will alter the therapeutic intervention required and will influence patient prognosis and duration of survival. This article examines the causes of clinical deterioration in patients with IPF and the diagnostic procedures for assessing disease-associated complications and staging IPF progression.

References (75)

  • GL Snider

    Interstitial pulmonary fibrosis: clinical features, natural history, and complications

    Semin Respir Med

    (1984)
  • AS Patchefsky et al.

    Desquamative interstitial pneumonia: relationship to interstitial fibrosis

    Thorax

    (1973)
  • DE Stover et al.

    Bronchoalveolar lavage in the diagnosis of diffuse pulmonary infiltrates in the immunosuppressed host

    Ann Intern Med

    (1984)
  • EA Gaensler et al.

    Radiographic-physiologic-pathologic correlations in interstitial pneumonias

    Prog Respir Res

    (1975)
  • LC Watters 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)
  • BR Line et al.

    Gallium-67 citrate scanning in the staging of idiopathic pulmonary fibrosis: correlation with physiologic and morphologic features and bronchoalveolar lavage

    Am Rev Respir Dis

    (1978)
  • M Turner-Warwick et al.

    Antibodies in some chronic fibrosing lung diseases I. Non organ-specific autoantibodies

    Clin Allergy

    (1971)
  • CF Pantin et al.

    Measures of the inflammatory response in cryptogenic fibrosing alveolitis

    Am Rev Respir Dis

    (1988)
  • RG Crystal et al.

    Interstitial lung diseases of unknown cause

    RG Crystal et al.

    Interstitial lung diseases of unknown cause

  • TE King

    Idiopathic pulmonary fibrosis

  • RG Crystal et al.

    Interstitial lung disease: current concepts of pathogenesis, staging and therapy

    Am J Med

    (1981)
  • CB Carrington et al.

    Natural and treated course of usual and desquamative interstitial pneumonia

    N Engl J Med

    (1978)
  • JL Livingstone et al.

    Diffuse interstitial pulmonary fibrosis: a clinical, radiological, and pathological study based on 45 patients

    Q J Med

    (1964)
  • M Turner-Warwick et al.

    Cryptogenic fibrosing alveolitis: clinical features and their influence on survival

    Thorax

    (1980)
  • BHR Stack et al.

    The prognosis of cryptogenic fibrosing alveolitis

    Thorax

    (1972)
  • RH Winterbauer et al.

    Diffuse interstitial pneumonitis: clinicopathologic correlations in 20 patients treated with prednisone/azathioprine

    Am J Med

    (1978)
  • PH Wright et al.

    Cryptogenic fibrosing alveolitis: assessment by graded trephine lung biopsy histology compared with clinical, radiographic, and physiological features

    Br J Dis Chest

    (1981)
  • JI Kennedy et al.

    Pulmonary hypertension in the interstitial lung diseases

    Chest

    (1985)
  • B McLees et al.

    Correlative studies of pulmonary hypertension in idiopathic pulmonary fibrosis

    Am Rev Respir Dis

    (1977)
  • EJ Campbell et al.

    Idiopathic pulmonary fibrosis

    Arch Intern Med

    (1981)
  • RA Matthay et al.

    Cardiovascular function in cor pulmonale

  • VU Lutwyche

    Another presentation of fibrosing alveolitis and alveolar cell carcinoma

    Chest

    (1976)
  • R Haddad et al.

    Idiopathic diffuse interstitial pulmonary fibrosis (fibrosing alveolitis), atypical epithelial proliferation and lung cancer

    Am J Med

    (1968)
  • SW Cosgriff

    Thromboembolic complications associated with ACTH and cortisone therapy

    JAMA

    (1951)
  • PD Wagner et al.

    Distribution of ventilation-perfusion ratios in patients with interstitial lung disease

    Chest

    (1976)
  • AS Fauci et al.

    Glucocorticosteroid therapy: mechanisms of action and clinical considerations

    Ann Intern Med

    (1976)
  • Y Sachor et al.

    Increased incidence of pulmonary tuberculosis in chronic interstitial lung disease

    Thorax

    (1989)
  • Cited by (327)

    • Chronic diffuse lung diseases

      2022, Practical Pulmonary Pathology: A Diagnostic Approach
    • Rethinking Idiopathic Pulmonary Fibrosis

      2021, Clinics in Chest Medicine
    View all citing articles on Scopus

    This work was supported in part by SCOR Grant HL27353 and Training Grant HL07085, National Heart, Lung and Blood Institute.

    View full text