Systemic sclerosis, also known as systemic scleroderma, is a long-term autoimmune condition in which the immune system becomes overactive, small blood vessels are damaged, and tissue thickens in the skin and sometimes in organs inside the body [1,4].
This page provides general information. It does not replace advice from a doctor or another qualified healthcare professional.
Systemic sclerosis, also called systemic scleroderma, is a long-term autoimmune condition. Autoimmune means the immune system, which normally fights infection, attacks the person’s own tissue [1,2,4].
Three processes happen together [9]. The immune system becomes overactive. Small blood vessels are damaged and narrowed. Connective tissue, the material that holds the body together, makes too much collagen, the protein that gives tissue its strength [1,2]. That build-up is fibrosis, meaning scar-like thickening of tissue [1]. It affects the skin and can affect organs inside the body, most often the lungs, gut, heart and kidneys [4].
Localized scleroderma is a different condition. Scleroderma is an umbrella name, and the two are often confused. Localized scleroderma, also called morphoea, affects the skin and the tissue directly under it, not internal organs, so it is not life-threatening, and patches often settle by themselves [1,2,10].
Systemic sclerosis, the form that can also involve blood vessels and organs, has two main types [1,2]:
Prevalence means how many people have a condition at one time; incidence means how many are newly diagnosed each year. The most complete worldwide review pooled 61 prevalence and 39 incidence studies, arriving at about 17.6 people per 100,000 (confidence interval 15.1 to 20.5) and about 1.4 new diagnoses per 100,000 a year [7]. Orphanet, the European rare disease database, gives a broader band of 1 to 5 per 10,000 [4].
Individual studies ranged from 3.1 to 144.5 per 100,000. Pooled figures were higher in North America (25.9 per 100,000) and Oceania, meaning Australia and New Zealand (23.8), than Europe (14.8) or Asia (6.8). No prevalence study from Africa was found at all [7].
Part of that gap is real and part is measurement: studies using insurance codes gave higher figures than those using clinical criteria [7]. Countries with less access to specialist testing likely undercount, and every figure here counts diagnosed people only.
Sex is the clearest pattern: 28.0 per 100,000 in women against 6.0 in men [7]. Onset is usually between 30 and 50, and children are rarely affected [1,2].
Nobody knows what causes systemic sclerosis [2,4]. Research points to a combination of factors rather than a single trigger.
Genes are involved. A gene is an instruction inside a cell, and certain genes appear to make the condition more likely and to influence which form develops. It is not passed from parent to child the way some genetic conditions are, though close relatives carry a somewhat higher risk [2,14].
Exposure to some chemicals, including silica, solvents and hydrocarbons, has been linked to it [2,4]. Hormones may play a part, one explanation for the sex difference. In the United States the condition is more common in African American people and tends to begin earlier. Nothing a person did caused it, and there is no way to prevent it [2].
Symptoms vary a great deal from person to person [10,14].
Raynaud’s phenomenon is usually the first sign: fingers and sometimes toes change color in the cold or with stress, turning pale, then blue, then red. In the diffuse form other signs follow within months; in the limited form they can follow years later [4].
Other common symptoms are [1,2,4]:
Two lung problems cause most deaths linked directly to the condition, and regular checks mean both can be treated early [3]. Interstitial lung disease, inflammation and scarring in lung tissue, affects around 40 percent of people to a degree that shows clinically [12]. Pulmonary arterial hypertension, raised blood pressure in the vessels carrying blood to the lungs, occurs in roughly 8 to 17 percent [13]. One European study attributed 35 percent of deaths caused directly by the condition to lung fibrosis and 26 percent to pulmonary arterial hypertension [8]. See our pulmonary hypertension guide.
When to seek urgent help. Scleroderma renal crisis is uncommon and a medical emergency. Blood pressure rises suddenly and steeply, usually above 150/85, and kidney function can fall quickly [3]. Contact a doctor straight away if a home reading is much higher than usual, or if there is a bad headache, changed vision, a seizure or sudden breathlessness. Treated promptly, kidney injury can often be prevented. Risk is highest in the first year of rapidly progressing diffuse disease, and after high doses of steroid medicines [11].
No single test gives a yes or no answer. Diagnosis comes from the whole picture: symptoms, examination and blood tests, usually led by a rheumatologist, a specialist in joint and connective tissue conditions [3,16].
Blood tests look for antibodies that mistakenly target the body’s own tissue [3]. An antinuclear antibody test, shortened to ANA, is a common first step [16]. Three more specific antibodies help sort out which form someone has: anti-centromere, anti-topoisomerase I, also written anti-Scl-70, and anti-RNA polymerase III [6]. The last is strongly linked to renal crisis, so its presence changes how closely blood pressure is watched [11]. A blood test alone cannot diagnose it, since these antibodies appear for other reasons [3].
Nailfold capillaroscopy is a painless look under magnification at the tiny blood vessels at the base of the fingernail, where enlarged vessels, missing vessels or small bleeds are a recognized sign [6]. A small skin sample may be taken, and skin thickening is scored to track change [16].
Other tests check for organ involvement: a detailed lung scan called high-resolution computed tomography, pulmonary function tests, an echocardiogram and blood pressure checks [3,4,13].
A scoring system agreed internationally in 2013 decides who joins a study rather than diagnosing someone in clinic [6]. Diagnosis is often delayed, since the condition develops slowly and takes different forms [16].
There is no cure [1,10]. Treatment is directed at each affected part of the body rather than one medicine for the whole condition. European recommendations updated in 2023 cover eight areas of the body, and care works best with a specialist team [5,17].
Kidneys. An ACE inhibitor, a blood pressure medicine, is started immediately when renal crisis is diagnosed. Anyone on steroid medicines should have blood pressure checked regularly [5].
Raynaud’s phenomenon and finger sores. A calcium channel blocker, usually nifedipine, is tried first, then PDE5 inhibitors, tablets that widen blood vessels, and iloprost by drip for severe symptoms. Bosentan is used to reduce new finger ulcers [5].
Lungs. For interstitial lung disease, mycophenolate mofetil, cyclophosphamide, rituximab, tocilizumab and nintedanib, an antifibrotic tablet, are recommended, nintedanib either alone or with mycophenolate. United States regulators have approved tocilizumab for this use. For pulmonary arterial hypertension, a PDE5 inhibitor plus an endothelin receptor blocker is recommended from the start [5].
Skin, joints and muscles. Methotrexate, mycophenolate mofetil and rituximab are considered for skin thickening, and tocilizumab in early inflammatory diffuse disease. Methotrexate also helps joint and muscle involvement [5].
Digestive system. Proton pump inhibitors, which reduce stomach acid, treat reflux and help protect the food pipe. Medicines that help the gut move food along are used for related symptoms, and rotating antibiotics when too many bacteria grow in the gut [5].
Rapidly progressive disease. For a small, carefully selected group with early diffuse disease and a poor outlook, without advanced heart or lung involvement, an autologous haematopoietic stem cell transplant may be considered: a person’s own blood stem cells are collected, intensive immune-suppressing treatment is given, then the cells are returned. Trial results have been clearly positive, and the risk of dying from the treatment itself must be weighed carefully. Availability and cost differ between countries [5].
Care usually continues for years with a specialist team, following a plan agreed with the person themselves. Physiotherapy and occupational therapy help with movement and daily tasks [3,17].
Keeping warm helps a great deal with Raynaud’s phenomenon: layers, gloves and socks, avoiding cold and damp. Stopping smoking matters, since nicotine narrows blood vessels. Moisturizers and gentle soaps help the skin, and exercise supports circulation. For reflux, smaller more frequent meals help, with soft food chewed well, staying upright for around three hours after eating, and raising the head of the bed on blocks [3].
Measuring blood pressure at home at least every other day gives early warning of renal crisis. Steroid medicines and painkillers such as ibuprofen are best kept to a minimum where possible, on medical advice [11].
Pregnancy needs planning, since conceiving can be harder and there is a slightly higher risk of miscarriage and early birth, though a pregnancy planned during a settled period can go well [1]. Support groups help many people, and a mental health professional can help with the weight of a long illness [3].
Clinical trials test whether a treatment works and is safe. It helps to take this in steps.
Be clear on:
Every trial has rules about who can take part, called eligibility criteria: some describe who can join, others who cannot. For a systemic sclerosis study they might include a diagnosis meeting the 2013 classification criteria, how long ago the first symptom other than Raynaud’s phenomenon appeared, a skin thickening score and lung function within set limits, and contraception for some treatments.
trialport’s medifit helps people consider information related to possible medical suitability. It does not diagnose a condition, confirm eligibility or replace formal screening by the study team.
Explore systemic sclerosis clinical trials through trialport
A study can look right on paper and still be hard in practice. Worth thinking through:
trialport’s readifit helps people reflect on their understanding, motivation, time, routines, support, emotions and practical arrangements.
Useful questions for a research team:
Taking part is voluntary. A person can ask questions, speak with people they trust and choose not to participate.
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Research is active on several fronts [5]:
Study status checked: 3 August 2026. Research moves and what is studied changes, so this list will date. For current information, search at app.trialport.com.
In the United States, the National Scleroderma Foundation runs local chapters, support groups, a peer mentor program and a support line [14]. The Scleroderma Research Foundation funds research and publishes clear material on individual complications, including scleroderma renal crisis [10,11]. NIAMS covers the condition and practical daily steps [2,3].
In the United Kingdom, Scleroderma & Raynaud’s UK is the only charity dedicated to both conditions, with a free helpline, support groups, a specialist finder and quality-marked information in several languages [15,17]. The NHS page on scleroderma is a good short starting point [1].
Elsewhere coverage is uneven. Orphanet keeps directories of patient organizations and expert centres by country [4].
Is scleroderma the same as systemic sclerosis?
Not quite. Scleroderma is the umbrella name. Localized scleroderma affects the skin and the tissue just under it, not internal organs. Systemic sclerosis can also affect blood vessels and organs [1,2].
How serious is it?
It varies enormously. Some people have mild skin changes and Raynaud’s phenomenon for years; others develop lung, heart or kidney involvement needing close treatment. Lung fibrosis and pulmonary arterial hypertension cause most deaths linked directly to the condition, and both can be found early [8].
Is it inherited?
Not directly. It is not passed from parent to child the way some genetic conditions are, though close relatives have a somewhat higher risk [2,14].
Can it be cured?
No, though treatment aimed at each affected part of the body eases symptoms and slows damage. People with the condition are living longer, fuller lives than they once did [1,10].
Is it related to lupus?
They are separate autoimmune conditions sharing some features, and doctors consider lupus when working out a diagnosis [4]. See our systemic lupus erythematosus guide.
Written by: trialport editorial team
Reviewed by: Keith Berelowitz, Founder and CEO, trialport
Reviewed on: 3 August 2026
Next review due: 3 August 2027
References last checked: 3 August 2026