Scleroderma is a connective tissue disorder characterized by skin thickening and fibrosis. It is rare, autoimmune and chronic.
It has an idiopathic cause and not contagious.
Early microvascular damage, mononuclear cell infiltrates and slowly developing fibrosis are the important features of the tissue lesions.
The leading causes of death are pulmonary fibrosis and pulmonary arterial hypertension.
In patients with heart failure with reduced ejection fraction (HFrEF) receiving angiotensin-converting-enzyme (ACE) inhibitors, high dosing confers benefits for the risk of death or hospitalization that are similar to that obtained with lower dosing, according to a systematic review and meta-analysis.