What is systemic sclerosis (scleroderma)?

Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality.

What is the difference between systemic sclerosis and diffuse cutaneous SSC?

In diffuse cutaneous SSc, the skin thickening can additionally involve the upper arms, thighs, and the trunk. It is possible to have Systemic Sclerosis without skin thickening (sine scleroderma), which is when a patient has the internal organ manifestations without the skin findings, but this is very rare.

Can systemic sclerosis affect the lungs and digestive system?

Limited systemic sclerosis can affect the lungs and the digestive system too. Systemic sclerosis is not to be confused with a separate condition called localised scleroderma, which just affects the skin. The word scleroderma, which is an older term, specifically means hard skin.

What are the complications of systemic sclerosis?

When systemic sclerosis causes connective tissue to harden this can stop muscles, blood vessels, joints and internal organs such as the lungs, working as well as they should. Most people with systemic sclerosis will only have mild symptoms. Rarely, it can cause serious problems with internal organs. These complications can be treated.

Is it possible to have systemic sclerosis without thickening of the skin?

It is possible to have Systemic Sclerosis without skin thickening (sine scleroderma), which is when a patient has the internal organ manifestations without the skin findings, but this is very rare.

What is a baseline investigation in systemic sclerosis?

Baseline investigations form part of the essential early investigation of systemic sclerosis. Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: application of a latent linear trajectory model.

How can we improve the management of systemic sclerosis?

Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis.