5th June 2025

Pathophysiology

The precise mechanism is unknown, but many models have been proposed. At the start of the disease, the immune system, endothelial cells and fibroblasts are all activated. A sub-population of hyper-proliferative fibroblasts is selected for, which are confirmed to be over-represented in scleroderma. These cells produce high levels of collagen. Activated T-cells contribute to disease by producing pro-fibrotic cytokines and downregulating cytokines that inhibit growth or synthesis.