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Systemic Sclerosis (Scleroderma)

Systemic Sclerosis (Scleroderma) - What it is

Scleroderma literally means “hard skin”. Scleroderma, also known as Systemic Sclerosis, is a chronic autoimmune condition in which the body’s immune system attacks its own tissues. Typically, abnormalities in the blood vessels, connective tissue and the immune system occur, resulting in inflammation and damage affecting the skin, digestive system, heart, lungs, muscles and joints.

scleroderma (systemic sclerosis) is when the body’s immune system attacks its own tissues

Whilst Scleroderma is not a common condition, it can have a profound impact on people and their lifespan. An estimated 20 to 200 people per million are affected worldwide, with the age of onset usually between 30 to 50 years. Women are 4 times more likely to develop the condition than men.

The body’s faulty immune system stimulates the fibroblast cells to produce excess collagen, causing fibrosis. There are two major types based on the extent of skin involvement that causes hardening, thickening and tightness of the skin: Limited Scleroderma and Diffuse Scleroderma with different patterns of involvement and symptoms.

  • In Limited Scleroderma, the skin fibrosis is less widespread and is confined to the fingers, hands, lower arms, lower legs and the face. Limited Scleroderma commonly starts slowly and Raynaud’s phenomenon often occurs many years before the skin becomes involved. One of the later manifestations may include heart / lung involvement called pulmonary arterial hypertension (increased blood pressure in the artery that carries blood from the heart to the lungs).
  • In Diffuse Scleroderma, the skin fibrosis is more widespread and has a much faster onset and progression than Limited Scleroderma. More importantly, people with Diffuse Scleroderma have a higher risk of developing internal organ involvement such as the heart, lungs and kidneys.

Systemic Sclerosis (Scleroderma) - How to prevent?

Systemic Sclerosis (Scleroderma) - Post-surgery care

Systemic Sclerosis (Scleroderma) - Other Information