Scleroderma

Overview

Scleroderma is a rare condition that causes the skin and sometimes other parts of the body to become thick, hard, and tight. It happens when the immune system attacks healthy tissue and triggers too much collagen, the protein that builds connective tissue. Scleroderma can affect only a few areas of skin or, in some people, it can affect internal organs such as the lungs, kidneys, and heart.

Scleroderma is rare. It is estimated that around 2.5 million people worldwide have the condition, but it is hard to know the exact number because some people have very mild symptoms.

Scleroderma can affect anyone at any age, but it is about four times more common in women than in men. It most often starts between the ages of 30 and 50, though children and older adults can also develop it.

Symptoms

Common symptoms

• Raynaud's phenomenon – fingers or toes turning white, blue, or purple in the cold and then throbbing when they warm up
• Thickening, hardening, and tightness of the skin, especially on the fingers, hands, and face
• Joint pain, stiffness, or swelling
• Tiredness (fatigue) that does not go away with rest
• Heartburn or difficulty swallowing
• Shortness of breath or a dry cough

Symptoms in children

• Children with scleroderma are more likely to have the localized form, which affects the skin rather than internal organs.
• They may have skin changes that affect one area, such as a limb or the face, and this can sometimes affect growth of that limb.
• They can also experience fatigue, joint pain, and Raynaud's phenomenon, though it is less common than in adults.

Symptoms in older adults

• Older adults may have more advanced skin tightness and a higher chance of internal organ involvement.
• They may also have other age-related health conditions that can make symptoms harder to recognise.
• Raynaud's phenomenon and swallowing problems can be more noticeable and may affect daily activities.

Causes

Main causes

• The exact cause of scleroderma is unknown.
• Doctors believe it happens when the immune system mistakenly damages healthy tissue and causes too much collagen to build up.
• A combination of genetic and environmental factors may trigger the disease, but it is not contagious and cannot be passed from person to person.

Risk factors

• Being female – women are affected more often than men
• Having a family history of scleroderma or other autoimmune diseases
• Certain environmental triggers, such as some chemicals or infections, may play a role, though this is not fully proven

When to see a doctor

See a doctor urgently if:

• If you have sudden breathlessness, chest pain, or any of the emergency symptoms listed above, call your local emergency number immediately.
• If you develop new or worsening symptoms that affect your daily life, contact your doctor as soon as possible.

Book a routine appointment if:

• If you notice persistent thickening or hardening of your skin, especially on your fingers or face
• If you have frequent heartburn, difficulty swallowing, or unexplained fatigue
• If you have Raynaud's phenomenon that is new or getting worse

Diagnosis

There is no single test for scleroderma. A doctor will ask about your symptoms, examine your skin, and may refer you to a rheumatologist (a specialist in conditions affecting joints and connective tissues). The diagnosis is usually based on a combination of your symptoms, physical signs, and test results.

Tests that may be done

• Blood tests to look for specific antibodies often found in people with scleroderma
• A skin biopsy, where a small sample of skin is taken and examined under a microscope
• Breathing tests and a chest X-ray or CT scan to check the lungs
• Blood pressure checks and other tests to check kidney and heart function

What to expect at your appointment

The diagnosis process can take time because scleroderma can look like other conditions. A specialist will work through the tests with you and explain what each one is for. You will likely have regular follow-up appointments to monitor your health.

Treatment

There is no cure for scleroderma, but there are many ways to manage the symptoms and prevent complications. Treatment is tailored to the type of scleroderma you have and which parts of your body are affected. The goal is to keep you comfortable, protect your organs, and help you stay active.

Self-care at home

• Keep your hands and feet warm to help with Raynaud's symptoms – wear gloves, warm socks, and avoid rapid temperature changes
• Quit smoking, because smoking makes blood vessel problems worse
• Protect affected skin from injury and use moisturisers to keep it soft
• Eat smaller, frequent meals to help with heartburn and swallowing problems
• Stay as active as you can – gentle movement and stretching can help keep joints flexible

Medical treatments

Doctors use different types of medicines to treat scleroderma, depending on your symptoms. These may include medicines to improve blood flow, reduce stomach acid, lower blood pressure, or calm the immune system. Some treatments are taken every day, while others are given at a clinic or hospital. Your healthcare team will recommend a plan that is right for you. Never start or stop any medication without your doctor's advice.

When is surgery considered?

Surgery is occasionally used for scleroderma, for example to help severe fingertip ulcers or to remove hard calcium deposits that form under the skin. But most people with scleroderma do not need surgery.

Living with this condition

Living with scleroderma can be challenging, but many people manage well with the right support. It helps to learn about the condition, pace yourself, and stay in close touch with your healthcare team. Build a routine that includes rest, activity, and time for things you enjoy.

Lifestyle tips

• Keep a warm environment at home and work to avoid Raynaud's flare-ups
• Do gentle stretching or yoga to keep your skin and joints flexible
• Ask for help with heavy tasks when you need it – you do not have to do everything alone
• Avoid stress, which can make symptoms worse. Find relaxation techniques that work for you

Diet and exercise

There is no special diet that treats scleroderma, but eating a balanced diet is important. If you have heartburn, eating smaller meals, avoiding large meals before lying down, and raising the head of your bed can help. Regular gentle exercise such as walking, swimming, or cycling can keep your joints and muscles strong. Always check with your doctor before starting a new exercise plan.

Mental health and emotional wellbeing

Living with a chronic condition can feel overwhelming at times and can affect your mood. It is normal to feel worried, frustrated, or low. Talk to your doctor if you are struggling, and consider counselling or support groups. If you are having thoughts of self-harm or are in crisis, contact your local mental health emergency service or call your local emergency number right away – you are not alone.

Prevention

Scleroderma cannot be prevented, because the cause is not fully understood. You cannot catch it and there is no vaccine against it. If you have a family history, you cannot stop it developing, but you can monitor for symptoms and seek help early.

Vaccines

It is important to stay up to date with recommended vaccinations, such as the flu vaccine and COVID-19 vaccine. These do not prevent scleroderma, but they help protect your lungs, which is especially important if scleroderma affects your breathing.

Screening programmes

There is no routine screening test for scleroderma in people without symptoms. However, if you have a close relative with scleroderma or another autoimmune disease, tell your doctor so they can be aware.

Complications

If left untreated

• Lung damage, including scarring of the lungs (pulmonary fibrosis) that can make breathing difficult
• Kidney damage from high blood pressure – this can sometimes happen suddenly
• Heart problems, including irregular heart rhythms and heart failure
• Severe swallowing problems that can lead to weight loss and malnutrition
• Infected skin ulcers on the fingertips or toes

Long-term outlook

The outlook for scleroderma varies a lot from person to person. Many people with localized scleroderma have a normal life expectancy and few restrictions. In systemic scleroderma, the condition can be more serious, but treatments have improved greatly in recent years. With early diagnosis and good care, most people can live a full and meaningful life. Your healthcare team is there to help you manage the condition and protect your long-term health.

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