Systemic Sclerosis
Systemic sclerosis is an autoimmune connective tissue disease
which causes thickening and hardening of the skin. This is why the
condition is also known as scleroderma (sclero = hard, derma =
skin). Other symptoms include joint pain and stiffness, tightening
of the facial skin, colour changes and sensitivity of your fingers
and toes to cold (Raynaud's phenomenon), white chalky lumps
developing under the skin (calcinosis), and digestive problems such
as heartburn and difficulty swallowing. Systemic sclerosis can also
cause problems with your internal organs, particularly heart,
kidneys and lungs, as well as causing high blood pressure.
The diagnosis of systemic sclerosis is based on your symptoms,
findings on physical examination, blood tests and sometimes a skin
biopsy. You may also have breathing tests, scans of your chest, a
heart scan and tests to examine your swallowing and stomach.
Systemic sclerosis affects everyone differently. For some
people, it comes on very gradually over years; for others it
progresses more quickly. Most people will need to have annual tests
to monitor for complications affecting the internal organs.
There is no cure for systemic sclerosis at present but there are
a range of medications that can help control the symptoms and
reduce the risk of complications. These include medications to help
improve circulation, painkillers and anti-inflammatory medications
to help with joint pain, and blood pressure medications to help
prevent problems with the kidneys and heart. If your lungs are
affected, steroid tablets and other medications to suppress the
immune system may be used.
Although some symptoms require specific medication, there are a
variety of self-help measures which are important in treating
systemic sclerosis. Stretching exercises are particularly important
and need to be done regularly to help keep your skin and joints
flexible. Our physiotherapists and occupational therapists will be
able to teach you these exercises. It is also essential to keep
warm from top to toe to help keep a good supply of blood flowing to
your skin and stopping ulcers from forming. Smoking reduces the
blood flow to the skin and can make Raynaud's symptoms worse so we
advise that all patients stop smoking.
Useful resources for patients:
Useful resources for health professionals:
- British Society for Rheumatology guidelines for the treatment
of systemic sclerosis
https://academic.oup.com/rheumatology/article/55/10/1906/2196591
- European League Against Rheumatism updated recommendations for
the treatment of systemic sclerosis 2017
https://ard.bmj.com/content/76/8/1327.long
