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Interstitial lung disease in systemic sclerosis.

Predrag Ostojic1, Marco Matucci Cerinic, Richard Silver

  • 1Institute of Rheumatology, University of Belgrade, Belgrade, Serbia. ostojic@net.yu

Lung
|August 25, 2007
PubMed
Summary
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Interstitial lung disease (ILD) affects 70% of systemic sclerosis (SSc) patients, often presenting as nonspecific interstitial pneumonia. Early intervention with cyclophosphamide shows promise for treating SSc-ILD.

Area of Science:

  • Pulmonary Medicine
  • Rheumatology
  • Pathology

Background:

  • Interstitial lung disease (ILD) is a significant complication in patients with systemic sclerosis (SSc).
  • Approximately 70% of SSc patients exhibit ILD at autopsy, with nonspecific interstitial pneumonia (NSIP) being the most frequent pathological finding.
  • The early stages of SSc-ILD involve microvascular injury and alveolitis.

Purpose of the Study:

  • To review the current literature on the pathogenesis, clinical features, diagnosis, and treatment of ILD in SSc patients.
  • To elucidate the key pathological mechanisms underlying SSc-ILD.
  • To assess the efficacy of diagnostic and therapeutic strategies for SSc-ILD.

Main Methods:

  • Literature review of studies on SSc-ILD.

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  • Analysis of pathological findings, including nonspecific interstitial pneumonia (NSIP).
  • Evaluation of diagnostic tools such as chest X-ray, HRCT, BAL, PFTs, lung biopsy, induced sputum, and 99mTc-DTPA clearance time.

    • Main Results:

    • Hallmarks of early SSc-ILD include endothelial lesions, coagulation protease activation (thrombin), fibroblast proliferation, and myofibroblast differentiation.
    • Standard diagnostic methods include imaging, lavage, lung function tests, and biopsy.
    • Novel diagnostic approaches like induced sputum and 99mTc-DTPA clearance time show potential utility.
    • Cyclophosphamide demonstrated effectiveness in the early phase of SSc-ILD.

    Conclusions:

    • ILD is a prevalent and serious manifestation of SSc.
    • Understanding the pathogenesis involving microvascular injury and fibroblast activation is crucial.
    • Early diagnosis and treatment, potentially with cyclophosphamide, are important for managing SSc-ILD, although the efficacy of other immunosuppressants requires further investigation.