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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

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

D Tansey1, A U Wells, T V Colby

  • 1Department of Histopathology, Royal Brompton Hospital, London, UK.

Histopathology
|June 10, 2004
PubMed
Abstract

Insights

Non-specific interstitial pneumonia (NSIP) is the most common lung disease in connective tissue disorders (CTDs). Pulmonary issues can precede systemic symptoms in CTDs, suggesting a distinct disease process from idiopathic lung diseases.

Area of Science:

  • Pulmonary Medicine
  • Rheumatology
  • Pathology

Background:

  • Connective tissue disorders (CTDs) frequently involve the lungs.
  • Previous studies often predate the recognition of non-specific interstitial pneumonia (NSIP).

Purpose of the Study:

  • To review lung biopsies from patients with CTDs to determine the frequency of interstitial pneumonia patterns.
  • To assess the impact of these patterns on patient prognosis.

Main Methods:

  • Analysis of 54 lung biopsies from 37 patients with polymyositis/dermatomyositis (PM/DM), Sjögren's syndrome, rheumatoid arthritis, and systemic lupus erythematosus (SLE).
  • Categorization of interstitial pneumonia patterns, including NSIP and usual interstitial pneumonia (UIP).

Main Results:

  • NSIP was the most common pattern (41% of patients).
  • Prevalence varied by CTD: PM/DM often showed organizing pneumonia, rheumatoid arthritis showed follicular bronchiolitis, and Sjögren's syndrome showed chronic bronchiolitis.
  • Usual interstitial pneumonia (UIP) was rare (4 patients).
  • Overall mortality was 24%, with fibrotic NSIP being the most frequent associated pattern.
  • In 9 cases, lung disease preceded the CTD diagnosis.

Conclusions:

  • NSIP is the predominant interstitial pneumonia pattern in CTDs, differing from idiopathic interstitial pneumonias where UIP is more common.
  • Interstitial pneumonia patterns vary among CTDs and can overlap.
  • Mortality is comparable to idiopathic NSIP, and early pulmonary presentation may influence the classification of some idiopathic NSIP cases.

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