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
- interstitial lung disease - general article
- systemic sclerosis - general article
Siehe auch:
