Scleroderma-related interstitial lung disease (SSc-ILD)

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 . It can sometimes produce a rapidly progressive interstitial lung disease pattern .

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