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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Serologic evaluation in idiopathic interstitial pneumonias.

Spyridon A Papiris1, Konstantinos Kagouridis, Demosthenes Bouros

  • 12nd Department of Pneumonology, Athens Medical School, National and Kapodistrian University of Athens, Attikon University Hospital, Haidari, Attika, Greece.

Current Opinion in Pulmonary Medicine
|June 16, 2012
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Idiopathic interstitial pneumonias (IIPs) can be associated with connective tissue diseases (CTDs). Early serologic evaluation is crucial for distinguishing IIPs from CTD-associated interstitial lung disease (ILD) for appropriate management and prognosis.

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Area of Science:

  • Pulmonology
  • Rheumatology
  • Immunology

Background:

  • Distinguishing idiopathic interstitial pneumonias (IIPs) from interstitial lung diseases (ILDs) associated with connective tissue diseases (CTDs) is critical for accurate diagnosis and management.
  • CTDs can present with ILD, sometimes preceding or occurring years after definitive CTD diagnosis.

Purpose of the Study:

  • To review the diagnostic evaluation necessary to differentiate IIPs from CTD-associated ILDs.
  • To highlight the role of serology in this diagnostic process.

Main Methods:

  • Review of recent literature on the association between IIPs and CTDs.
  • Emphasis on serologic evaluation, including autoantibodies and inflammatory biomarkers.
  • Consideration of clinical clues and histopathologic features.

Main Results:

  • Growing evidence suggests that patients initially diagnosed with IIPs may later show evidence of CTDs, particularly nonspecific interstitial pneumonia.
  • ILD can be the initial or presenting manifestation of an undiagnosed or undifferentiated CTD.

Conclusions:

  • Interstitial lung diseases can occur independently or in conjunction with CTDs.
  • Accurate diagnosis of ILD associated with CTD is essential due to differences in natural history, pathobiology, treatment, and prognosis.
  • A comprehensive approach including serology, clinical assessment, and histopathology aids in diagnosis.