Systemic Sclerosis

Systemic sclerosis, also called scleroderma, is a rare autoimmune condition in which the immune system drives scarring in the skin, damage to small blood vessels, and in many people scarring in organs inside the body. It often begins with fingers that turn white or blue in the cold and skin on the hands that feels tight and shiny. What follows differs a great deal from one person to the next.

What systemic sclerosis is, and what it is not

Scleroderma is the broader word, and it covers more than one condition. Sclérodermie Québec separates localised scleroderma, which includes morphea and linear forms and does not affect internal organs, from systemic sclerosis, which can. Systemic sclerosis is then divided again: a limited cutaneous form, where skin changes stay on the hands, forearms, feet, lower legs, face and neck, and a diffuse cutaneous form, where they reach above the elbows and knees onto the upper arms, thighs, trunk or abdomen, and where organ involvement tends to be more common and more extensive.

So this is not a skin condition with one course and one outcome. A 2026 CMAJ review reports that gastrointestinal involvement affects about 90% of people, interstitial lung disease 25% to 50%, pulmonary arterial hypertension 5% to 10% and renal crisis 1% to 14%. Some people have limited skin changes and stable lungs for years. Others have organ involvement that needs close watching from the start.

Signs and symptoms

• Fingers and toes that turn white, blue or red and become painful in the cold or with stress, known as Raynaud phenomenon
• Skin on the fingers or hands that becomes tight, shiny and hard, sometimes puffy and swollen first
• Sores or ulcers on the fingertips, and small hard calcium lumps under the skin
• Heartburn, food sticking on the way down, bloating, constipation or diarrhoea
• Shortness of breath when you exert yourself, or a dry cough that does not settle
• Joint pain and stiffness, muscle weakness, and unusual tiredness
• Changes in the face and mouth, including a smaller mouth opening and dryness

Which of these appear, in what order, and how fast they change varies widely, and one person can be stable for long stretches and then not.

What raises the risk

Some things cannot be changed:

• Being female. Women outnumbered men about four to one in the Quebec data, and Arthritis Society Canada reports scleroderma affects women three to five times more often than men
• Age. The average age at diagnosis in the Quebec study was 57.3 years, though systemic sclerosis can begin in childhood
• Genetic background, which the CMAJ review says accounts for about 18% of risk
• Ancestry. A 2026 systematic review found higher rates of diffuse cutaneous disease, interstitial lung disease and reduced survival among people of African descent, Indigenous American and some South Asian populations

Others can often be worked on, usually with support from a health care team:

• Workplace exposure to respirable crystalline silica, in work such as construction and mining. In the Canadian Scleroderma Research Group cohort of 1,439 people, about 7% overall and 20% of the men reported silica exposure, which went with younger age at diagnosis, worse organ involvement and higher mortality
• Workplace exposure to organic solvents, which the CMAJ review lists alongside silica
• Smoking, which the CMAJ review names with cardiovascular risk reduction among the general steps in care, because of the effect on blood vessels

How systemic sclerosis is diagnosed in Canada

There is no single test that confirms scleroderma, as Arthritis Society Canada notes, so the diagnosis rests on the pattern of findings. Most people in Canada are seen first by a family doctor or nurse practitioner and referred to a rheumatologist, often with a dermatologist involved. Systemic sclerosis is the most common cause of secondary Raynaud phenomenon, so a new Raynaud pattern in an adult is often what starts the assessment. The CMAJ review describes initial testing with antinuclear antibody, complete blood count, C-reactive protein and serum protein electrophoresis, alongside examination of the skin and hands. Because the lungs, heart, gut and kidneys can be involved, baseline spirometry and echocardiography are done at diagnosis, high-resolution CT of the chest is used for people at higher risk, and blood pressure, creatinine and urinalysis are checked each year. A skin biopsy is sometimes taken.

Treatment and day-to-day management

Clinical research for Systemic Sclerosis is enrolling. See the current studies ↓

Care is organised around the organs involved rather than around one medicine, and usually means a rheumatologist working with other specialists over years. Depending on your situation, a health care team may discuss:

• Medicines for Raynaud phenomenon and fingertip ulcers, including extended-release nifedipine, together with practical warmth measures such as heated mittens
• Medicines for reflux and other gut symptoms, including proton pump inhibitors
• Immune-targeting and antifibrotic medicines when the skin and lungs are involved. The 2023 American Thoracic Society guideline on systemic sclerosis interstitial lung disease reviewed cyclophosphamide, mycophenolate, rituximab, tocilizumab, nintedanib and pirfenidone, alone and in combination
• Regular monitoring, including annual blood pressure, creatinine and urinalysis to watch for renal crisis, plus lung function testing and heart assessment
• Non-drug support, including physiotherapy, occupational therapy, hand and skin care, help with swallowing and nutrition, smoking cessation and cardiovascular risk reduction

What fits one person will not fit another, and the plan usually changes over time. These are decisions for you and your own specialist team, and this page is not a recommendation about any of them.

When to talk to a doctor

• New or worsening shortness of breath, or a new dry cough, needs prompt assessment. Call 911 or go to an emergency department if breathing becomes difficult at rest.
• New high blood pressure, a sudden drop in how much urine you are passing, or a rapid rise in creatinine on a blood test needs same-day assessment, because renal crisis in systemic sclerosis is a medical emergency.
• Rapidly thickening skin, or a fingertip sore that is not healing, turning black or becoming very painful, should be assessed urgently rather than watched at home.

Why clinical research matters for systemic sclerosis

Arthritis Society Canada states there is currently no cure for scleroderma and that treatment is aimed at controlling symptoms. The Canadian numbers show why that matters: incidence in Quebec rose by an average of 3.94% a year, and although the standardised mortality ratio fell from 4.18 in 1996 to 2.69 in 2019, deaths were still about 2.7 times the expected rate. A 2025 review in Nature Reviews Rheumatology describes systemic sclerosis as an area of very active drug development, with many medicines using different mechanisms either in clinical trials or about to enter them, aimed at slowing fibrosis. Cell-based approaches are also being studied, including haematopoietic stem cell transplantation, mesenchymal stem cells, regulatory T cells and CD19-directed CAR T cell therapy, tried so far in small numbers of people with progressive disease. Reversing lung scarring that is already established remains one of the hardest problems. A 2026 systematic review points to earlier detection and more standardised care in expert centres as separate gaps.

Clinical studies are how those questions get settled. Taking part is voluntary, you give informed consent before anything begins, and you can withdraw at any time without affecting the care you get from your own doctor. In Scleroderma Canada’s survey of 358 Canadians, 55% had considered joining a clinical trial and 24% had done so. Joining a study does not mean giving up your current specialist, and no one can say in advance whether a study will help you personally.

Learn more from Canadian sources

• Scleroderma Canada
• Sclérodermie Québec: the different forms of scleroderma
• Canadian Scleroderma Research Group
• Arthritis Society Canada: scleroderma