Interstitial lung disease associated with systemic sclerosis

Interstitial lung disease associated with systemic sclerosis (SSc-ILD) is one of the important pulmonary manifestations of systemic sclerosis.  It usually gives a NSIP type pattern with an UIP pattern occurring less commonly 7. It can sometimes produce a rapidly progressive interstitial lung disease pattern 1.

Pathology

Exact pathogenesis is not fully understood at time of writing although three steps are thought to play a role which include

  • persistent and repeated bouts endothelial cell injury
  • activation of innate and adaptive immunity
  • fibroblast recruitment/activation, which then results in accumulation of extracellular matrix and scarring

Antibody markers

  • antinuclear antibodies (ANA): almost all SSc-ILD patients are positive
  • SSc-specific antibodies
    • anti-topoisomerase I (anti-topo I)  antibodies: frequently positive
    • anti-Th/To antibodies: frequently positive
    • anti-centromere antibodies: uncommonly positive

See also

Article information

rID: 81267
System: Chest
Synonyms or Alternate Spellings:
  • Interstitial lung disease in systemic sclerosis
  • Scleroderma interstitial lung disease
  • SSc-ILD
  • Scleroderma-associated interstitial lung disease
  • Scleroderma-related interstitial lung disease
  • Scleroderma-related interstitial lung disease (SSc-ILD)
  • Interstitial lung disease associated with systemic sclerosis (SSd-ILD)sD

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Cases and figures

  • Case 1
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