An uncommon disorder characterized by the thickening and scarring of multiple organ systems.
Systemic scleroderma is an advancing type of scleroderma, a rare autoimmune condition where the immune system targets the body’s own tissues. This disease can impact multiple organs and tissues, notably including the skin, arteries, kidneys, lungs, heart, digestive system, and joints. Women are three times more likely than men to develop systemic sclerosis, and it most commonly emerges between the ages of 30 and 50.
The severity of the disease can differ among individuals. It often starts with symptoms characteristic of Raynaud’s phenomenon, where fingers and toes turn white, lose sensation, and become painful in cold conditions. This is usually followed, sometimes years later, by the skin on the face, hands, forearms, and feet becoming thick and tight. In more severe instances, internal organs can also be affected. Symptoms might include difficulties in swallowing or heartburn due to damage to the esophagus. Pulmonary hypertension, or elevated blood pressure in the arteries that serve the lungs, may also occur, sometimes as long as 15 years after the disease first appears.
Systemic sclerosis typically advances quickly in the initial years before slowing down or even halting in its progression. For a small percentage of individuals, the decline is swift and may result in death due to heart, respiratory, or kidney failure. While there’s no cure for systemic sclerosis, symptom relief is often achievable through medication or physiotherapy.