Systemic sclerosis (scleroderma) explained

What systemic sclerosis is

Systemic sclerosis is an autoimmune condition, meaning the immune system, which normally fights infection, mistakenly causes inflammation and damage in the body's own tissues. In this condition three things go wrong together: the immune system is overactive, small blood vessels become narrowed and damaged, and the body lays down too much of a fibrous protein called collagen, which makes tissues thicken and harden. This scarring can affect the skin, making it tight and shiny, and can also affect internal organs. The word scleroderma literally means hard skin. There are different patterns — some mainly involve the skin of the hands, face and lower arms and legs, while others involve more widespread skin and a higher chance of organ problems. It is not contagious, not a cancer, and not caused by anything the person did.

The symptoms it causes

Symptoms depend on which tissues are affected. Very often the first sign is Raynaud's, where the fingers turn white, then blue, then red and become painful in the cold as small vessels clamp down. The skin of the fingers and hands may become tight, swollen and hard, and the face can feel taut. People may notice small dilated blood vessels on the skin, calcium lumps under the skin, joint stiffness and aching, and sores at the fingertips. When internal organs are involved, there may be heartburn and swallowing difficulty from the gullet, bloating and bowel changes, breathlessness or a dry cough if the lungs are affected, and, less commonly, problems with the heart or kidneys. Tiredness is common. Because the mix of features differs so much between people, the condition can look quite different from one person to the next.

How it is diagnosed and monitored

Systemic sclerosis is usually diagnosed by a specialist, often a rheumatologist, who puts together the pattern of skin changes, Raynaud's and other features with examination and tests. Blood tests can detect particular antibodies that are linked to the condition and help judge which pattern a person has and their risks. A close look at the tiny blood vessels at the base of the fingernails, using a special magnifier, can show characteristic changes. Because internal organ involvement may be silent early on, regular monitoring is a key part of care: breathing tests and lung scans check the lungs, heart scans and tracings check the heart, blood pressure and kidney checks are important, and other tests assess the gut. This ongoing surveillance aims to catch any organ problems early, when they can be treated most effectively.

How it is treated

There is no single cure, but a great deal can be done to control symptoms, protect organs and improve quality of life, and treatment is tailored to each person's pattern. Raynaud's is helped by keeping warm, avoiding cold and, if needed, medicines that widen the blood vessels. Skin and joint symptoms are managed with moisturising, physiotherapy and, in some cases, medicines that calm the immune system. Reflux and gut symptoms are treated with acid-lowering medicines and dietary measures. When the lungs, heart or kidneys are affected, specific treatments are used, and blood-pressure control is especially important to protect the kidneys. Medicines that dampen the immune system may be used for more active disease. Care is usually shared between a specialist team and the GP, and regular review allows treatment to be adjusted as the condition changes over time.

Living with systemic sclerosis

Living well with systemic sclerosis means combining medical care with practical self-management. Keeping warm and protecting the hands from cold reduces Raynaud's attacks, and careful skin care and gentle exercise help keep skin and joints supple. Stopping smoking is particularly important, as smoking harms the small blood vessels. Eating smaller, more frequent meals and staying upright after eating can ease reflux. Pacing activities helps with fatigue. Attending monitoring appointments is vital, because catching lung, heart or kidney involvement early makes treatment more effective; report new breathlessness, chest pain, marked swelling, a big change in blood pressure symptoms or worsening fingertip sores promptly. Emotional support matters too, as living with an unpredictable long-term condition can be hard. With good specialist care, monitoring and self-care, many people manage systemic sclerosis and maintain a good quality of life.