Pathogenesis (disease development)
The exact causes of scleroderma have not yet been determined. What has been proven so far are genetic factors (HLA associations in systemic scleroderma) and pathologic (pathological) autoimmunologic processes. Thus, it is known that immunocompetent cells (T cells (belong to the most important cell groups of the cellular immune defense), macrophages (“scavenger cells”)), fibroblasts (connective tissue cells), endothelial cells (cells of the inner wall of blood vessels) and their mediators (messenger substances) are mainly involved in the development of systemic scleroderma.
In chronic cutaneous circumscribed scleroderma, dysfunction of skin fibroblasts with excessive formation of collagen and ground substance, as well as trauma (injury), are considered as trigger and localization factors.
In systemic scleroderma, vasculopathy (group of primarily non-inflammatory vascular diseases of various causes) and an early inflammatory and later fibrotic phase (early phase associated with inflammation and late phase associated with pathological proliferation of connective tissue) occur: This leads to fibrosis of the skin and internal organs; mainly affected are the gastrointestinal tract/gastrointestinal tract (in 90% of cases is the esophagus), lungs (in 48% of cases), heart (in 16% of cases) and kidneys (in 14% of cases).
Etiology (causes)
Biographic causes
- Genetic burden from parents, grandparents (HLA associations in systemic scleroderma).
