Systemic Sclerosis

Also known as: Scleroderma

An autoimmune disease that causes thickening and hardening of the skin and can affect blood vessels, lungs, heart, kidneys and gut.

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Systemic Sclerosis

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How it works · Autoimmunity

The immune system protects you

B cells make antibodies, Y-shaped proteins that tag germs so the rest of the immune system can clear them.

What it is

Systemic sclerosis combines autoimmunity, damage to small blood vessels and excess collagen (fibrosis). Limited disease affects the hands, face and lower arms, while diffuse disease spreads more widely and more often involves organs.

Symptoms

Raynaud’s phenomenon, puffy fingers, tight shiny skin, finger ulcers, heartburn and swallowing problems, shortness of breath, joint pain and fatigue.

How it’s diagnosed

ANA plus specific antibodies (anti-centromere, anti-Scl-70, anti-RNA polymerase III), nailfold capillaroscopy, and baseline lung function tests, chest CT and heart ultrasound.

Treatment

Treatment targets each organ: vasodilators for Raynaud’s, acid suppression for reflux, mycophenolate or tocilizumab for lung and skin disease, nintedanib for lung fibrosis, and blood pressure medicines to protect the kidneys.

Living with it

Yearly lung and heart screening is important. Check blood pressure at home and report sudden rises. Hand exercises and moisturizers help skin tightness.

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Dermatomyositis →

At a glance