Interstitial lung disease associated with systemic sclerosis

Last revised by Yuranga Weerakkody on 15 May 2023

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.

The 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

  • 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

Some authors describe a four corners sign in terms of distribution of disease as being a characteristic feature 8.

ADVERTISEMENT: Supporters see fewer/no ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.