Chest
Volume 136, Issue 1, July 2009, Pages 23-30
Journal home page for Chest

Original Research
Interstitial Lung Disease
Pathologic and Radiologic Differences Between Idiopathic and Collagen Vascular Disease-Related Usual Interstitial Pneumonia

https://doi.org/10.1378/chest.08-2572Get rights and content

Background

Patients with usual interstitial pneumonia (UIP) associated with collagen vascular disease (CVD) have been reported to have a better prognosis than those with idiopathic pulmonary fibrosis with a UIP pattern (IPF/UIP) seen on histology. The aim of this study was to evaluate the pathologic and radiologic differences between the two conditions and their relationship with clinical outcome.

Methods

A retrospective review of 100 patients (CVD-UIP, 39 patients; IPF/UIP, 61 patients) with UIP pattern diagnosed by surgical lung biopsy at one tertiary referral center.

Results

The median follow-up period was 34.4 months. The CVD-UIP group was younger, included more women and nonsmokers, and showed better survival than the IPF/UIP group. Pathologically, CVD-UIP patients had fewer fibroblastic foci and smaller honeycombing (HC) spaces with higher germinal centers and total inflammation scores than IPF/UIP patients. Radiologically, CVD-UIP patients had a lower emphysema score and more likely a nontypical UIP pattern without HC. The germinal centers score was the best distinguishing feature between CVD-UIP and IPF/UIP patients (odds ratio, 2.948; p = 0.001) and was marginally related to survival (p = 0.076). The HC score (hazard ratio [HR], 1.134; p < 0.001), total lung capacity (TLC) [HR, 0.932; p = 0.004], and age (HR, 1.052; p = 0.017) were significant predictors of survival in all patients with UIP histology, regardless of the presence of CVD. Among IPF/UIP patients, those with positive autoantibodies were pathologically more similar to CVD-UIP than to IPF/UIP without autoantibodies, despite no difference in survival between them.

Conclusions

The germinal centers score was the best discriminative between CVD-UIP and IPF/UIP patients; it was of marginal prognostic significance. Age, TLC, and HC score were independent prognostic factors in all patients with UIP histology.

Section snippets

Study Populations

UIP was diagnosed in a total of 320 patients (IPF/UIP, 272 patients; CVD-UIP, 48 patients) by surgical lung biopsy at the Asan Medical Center in South Korea from August 1991 to December 2007. Idiopathic pulmonary fibrosis was diagnosed according to the American Thoracic Society/European Respiratory Society consensus classification,13 and individual CVDs were diagnosed in patients according to the criteria of the corresponding societies.14, 15, 16, 17, 18, 19, 20 All patients with IPF/UIP lacked

Comparison of Clinical Findings Between CVD-UIP and IPF/UIP Patients

The mean age of the study population was 56.6 years, and 56.0% were men. The median follow-up period for all patients was 33.4 months. The underlying types of CVD were rheumatoid arthritis (n = 19), systemic sclerosis (n = 13), Sjögren syndrome (n = 3), undifferentiated connective tissue disease (n = 2), polymyositis (n = 1), and mixed connective tissue disease (n = 1). The CVD-UIP group was younger, and it included more women and nonsmokers compared with the IPF/UIP group (Table 1). However,

Discussion

In this study, we found that CVD-UIP and IPF/UIP patients show different pathologic features, despite having the same basic UIP pattern; CVD-UIP patients had more germinal centers and total inflammation with plasma cells and fewer fibroblastic foci and smaller HC spaces histologically compared with IPF/UIP patients. Radiologically, CVD-UIP patients showed a lesser extent of emphysema, higher prevalence of nontypical UIP pattern without HC, and a tendency for less HC, at the same degree of lung

References (41)

  • J Fujita et al.

    Non-specific interstitial pneumonia as pulmonary involvement of systemic sclerosis

    Ann Rheum Dis

    (2001)
  • I Ito et al.

    Pulmonary manifestations of primary Sjögren's syndrome: a clinical, radiologic, and pathologic study

    Am J Respir Crit Care Med

    (2005)
  • DS Kim et al.

    The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia

    Sarcoidosis Vasc Diffuse Lung Dis

    (2002)
  • D Tansey et al.

    Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis

    Histopathology

    (2004)
  • JH Park et al.

    Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes

    Am J Respir Crit Care Med

    (2007)
  • Y Nakamura et al.

    Nonspecific interstitial pneumonia in collagen vascular diseases: comparison of the clinical characteristics and prognostic significance with usual interstitial pneumonia

    Sarcoidosis Vasc Diffuse Lung Dis

    (2003)
  • American Thoracic Society et al.

    American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias: this joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001

    Am J Respir Crit Care Med

    (2002)
  • Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee

    Preliminary criteria for the classification of systemic sclerosis (scleroderma)

    Arthritis Rheum

    (1980)
  • FC Arnett et al.

    The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis

    Arthritis Rheum

    (1988)
  • A Bohan et al.

    Polymyositis and dermatomyositis (first of two parts)

    N Engl J Med

    (1975)
  • Cited by (0)

    The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).

    View full text