Systemic Sclerosis


What is Systemic Sclerosis?

Systemic Sclerosis (SSc) (also known as scleroderma) is a long-term condition that causes thickening of the skin, but can also affect internal organs, too. It is an autoimmune condition, meaning that the immune system, which normally works to defend the body against infection and illness, starts to produce factors that damage healthy tissues.

Systemic sclerosis affects the body’s connective tissues, which lies under the surface of the skin and in and around the internal organs and blood vessels. Scleroderma leads to an excess of a protein called collagen in the connective tissues. Collagen is essential to hold tissues together; but too much can cause the body’s tissues to become stiff and thick.

It is divided into 2 main sub-conditions:


Causes of Systemic Sclerosis

Systemic sclerosis is a rare condition, with around 12,000 people in the UK having it. Females are 3 to 4 times more likely to develop scleroderma than males and the condition usually starts between the ages of 25-55 years.

The exact reason remains unknown, but we believe the immune system is to blame because it is associated with an excess of a protein called an autoantibody that is made by the immune system. These antibodies target the body’s healthy tissues.

It is thought that Systemic sclerosis is possibly triggered by environmental factors in those that have a predisposition due to genetic risk. Therefore having a family member with the condition can increase your risk.

As such other autoimmune diseases can be present with SSc, the most common being thyroid related or Sjögren’s syndrome.


Symptoms of Systemic Sclerosis

Systemic sclerosis can cause a variety of symptoms throughout the body, which differ between individuals.

The most common symptoms are:


How do we diagnose Systemic Sclerosis?

There is no single diagnostic test for scleroderma. Often, the key factor in making the diagnosis is the characteristic thickening of the skin, but other tests can be helpful, too.

Tests may include:



There is currently no cure for scleroderma. The aims of available treatments are to help control symptoms and treat complications.


Maintaining a healthy lifestyle is recommended, details on how to achieve this can be found here.

In order to monitor the disease clinic reviews are arranged alongside annual tests (lung function tests and ECHO) to check for early signs of complications. In addition, you should have your blood pressure and urine checked when you come to clinic.


Useful Links

Versus Arthritis:

Scleroderma and Raynaud’s UK: