Systemic sclerosis is a multisystem disorder with a high associated mortality. The hallmark abnormalities of the disease are in the immune system, vasculature, and connective tissue. Systemic sclerosis occurs in susceptible individuals and is stimulated by initiating events that are poorly understood at present. In order for the disease phenotype to appear there is dysfunction in the homoeostatic mechanisms of immune tolerance, endothelial physiology, and extracellular matrix turnover. The progression of disease is not sequential but requires simultaneous dysfunction in these normal regulatory mechanisms. Better understanding of the interplay of these factors is likely to contribute to improved treatment options.
Key points
- •
Systemic sclerosis is a multisystem disorder with a high associated mortality.
- •
The hallmark abnormalities of the disease are in the immune system, vasculature, and connective tissue.
- •
Systemic sclerosis occurs in susceptible individuals (as defined by genetic studies) and is stimulated by initiating events, although these initiating factors are poorly understood at present.
- •
In order for the disease phenotype to appear there is dysfunction in the homoeostatic mechanisms of immune tolerance, endothelial physiology, and extracellular matrix turnover.
- •
The progression of disease is not sequential but requires simultaneous dysfunction in these normal regulatory mechanisms.
- •
A better understanding of the interplay of these factors is likely to contribute to improved treatment options.
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
