Systemic sclerosis overview

Overview

Systemic sclerosis (also called scleroderma) is a rare condition where the body's immune system mistakenly attacks healthy tissue. This causes scar tissue (fibrosis) to build up, mainly in the skin but sometimes in internal organs like the lungs, heart, or kidneys. The skin becomes tight and hard, and blood vessels may be damaged.

No, systemic sclerosis is rare. It affects about 1 in 10,000 people worldwide.

It can occur at any age but is most common in people between 30 and 50 years old. Women are three to four times more likely to develop it than men.

Symptoms

Common symptoms

• Thickening and hardening of the skin, especially on the fingers, hands, and face
• Raynaud’s phenomenon – fingers or toes turn white or blue in the cold
• Small red spots on the skin (telangiectasias) – widened blood vessels
• Joint pain and stiffness
• Heartburn or trouble swallowing (due to scarring in the esophagus)

Symptoms in children

• Systemic sclerosis is very rare in children. When it does occur, symptoms may include skin tightening, joint pain, and Raynaud’s phenomenon. Growth and development can be affected.

Symptoms in older adults

• In older adults, systemic sclerosis may be mistaken for other age-related conditions. Symptoms like skin tightness, fatigue, and shortness of breath are common. Organ involvement (especially lungs) can be more severe.

Causes

Main causes

• The exact cause is unknown. It is an autoimmune disease where the immune system produces too much collagen (a protein that forms scar tissue). Genetics may play a role, but it is not directly inherited.

Risk factors

• Being female (much more common in women than men)
• Having a family history of autoimmune diseases (like lupus or rheumatoid arthritis)
• Certain environmental triggers, such as exposure to silica dust or some chemicals, though this is rare

When to see a doctor

See a doctor urgently if:

• If you have sudden chest pain, trouble breathing, or signs of a stroke (face drooping, arm weakness, slurred speech – call emergency services immediately).

Book a routine appointment if:

• If you notice persistent skin tightness, especially on your fingers, hands, or face
• If you have Raynaud’s phenomenon (fingers turning white/blue in cold) that bothers you
• If you have ongoing heartburn or trouble swallowing

Diagnosis

A doctor will take a detailed history and do a physical exam. They will check your skin, joints, and blood pressure. There is no single test for systemic sclerosis, but a combination of findings can suggest the diagnosis.

Tests that may be done

• Blood tests for certain antibodies (like anti-centromere or anti-topoisomerase I antibodies)
• Skin biopsy – taking a small sample of skin to look under a microscope
• Lung function tests to check how well your lungs work
• Echocardiogram (ultrasound of the heart) to see if the heart is affected
• X-rays or CT scans of the chest

What to expect at your appointment

Diagnosis can take time because symptoms develop slowly. Your doctor may refer you to a rheumatologist (a specialist in autoimmune diseases). Be prepared to discuss your symptoms in detail, and bring a list of any medicines you take.

Treatment

There is no cure for systemic sclerosis, but treatments can help manage symptoms and prevent complications. Treatment is tailored to each person based on which organs are involved and how severe the condition is.

Self-care at home

• Keep your skin moisturized with gentle, fragrance-free creams
• Protect your hands and feet from cold – wear warm gloves and socks
• Avoid smoking and limit alcohol, as they can worsen blood vessel problems
• Manage heartburn by eating smaller meals and not lying down right after eating

Medical treatments

Doctors may prescribe medicines to help with specific symptoms, such as medications to improve blood flow (for Raynaud’s), reduce acid reflux, or control blood pressure. For more severe cases, medications that suppress the immune system may be used. Physical or occupational therapy can help with joint stiffness.

When is surgery considered?

Surgery is rarely needed. In severe cases, if a finger or toe has gangrene (tissue death) that cannot be saved, amputation may be necessary. But this is very uncommon.

Living with this condition

Living with systemic sclerosis means managing symptoms day by day. You may need to adjust your daily activities to avoid cold, stress, and overexertion. Regular check-ups are important to monitor your skin, lungs, heart, and kidneys.

Lifestyle tips

• Stay active with gentle exercises like walking or stretching to keep joints flexible
• Eat a balanced diet with plenty of fruits, vegetables, and whole grains
• Get enough rest – fatigue is common with this condition

Diet and exercise

A healthy diet can help manage heartburn and maintain weight. Exercise, such as swimming or yoga, can improve circulation and reduce stiffness. Talk to your doctor before starting any new exercise program.

Mental health and emotional wellbeing

Living with a chronic illness can be stressful and may lead to anxiety or depression. It is important to talk to your doctor if you feel overwhelmed. If you are in crisis, reach out to a mental health helpline or your local emergency number.

Prevention

Systemic sclerosis cannot be prevented because the cause is not known. However, avoiding smoking and certain chemicals may reduce the risk of triggering the disease in people who are genetically prone.

Vaccines

It is important to stay up to date with vaccines, especially for flu and pneumonia, to prevent infections that can worsen your condition. Talk to your doctor about which vaccines are right for you.

Screening programmes

No routine screening tests exist for systemic sclerosis. If you have a family history of autoimmune diseases, tell your doctor, but routine testing is not recommended unless you have symptoms.

Complications

If left untreated

• Pulmonary fibrosis – scarring of the lungs that makes breathing difficult
• Pulmonary hypertension – high blood pressure in the lungs, straining the heart
• Kidney crisis – sudden high blood pressure and kidney damage
• Digestive problems – severe reflux, difficulty swallowing, malnutrition

Long-term outlook

The outlook for systemic sclerosis varies. Many people manage their symptoms well with treatment and have a good quality of life. More severe cases can lead to organ damage, but early detection and regular care improve outcomes. Research is ongoing, and new treatments are being developed.

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