Systemic Scleroderma

Disease Overview

Systemic scleroderma is a disease characterized by rapid growth of fibrous (connective) tissue that leads to scarring of skin and internal organs. Systemic scleroderma can affect almost any organ in the body, and there is a large variability of symptoms among affected people. One of the most common and earliest manifestations of the disease is Raynaud phenomenon, which involves blood vessel spasms (vasospasms) induced by cold temperature or stress. This can lead to temporary finger discoloration, numbness and pain and is also associated with the development of finger ulcers. Of note, Raynaud phenomenon also commonly occurs in healthy individuals.

Other manifestations of systemic scleroderma include muscle and joint pain, skin tightening and dilated blood vessels that can be seen through the skin (telangiectasias). Scarring of internal organs can also lead to gastrointestinal, pulmonary, cardiac and renal disease.

Approximately one in 10,000 individuals are affected. It is more common in females and most often develops around age 30 to 50.

Although systemic scleroderma cannot be cured, many of the symptoms can be treated. A timely diagnosis is important to ensure appropriate management of the disease and associated complications.

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Synonyms

• systemic sclerosis
• progressive systemic sclerosis

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Subdivisions

• limited cutaneous systemic sclerosis (lcSSc) also known as limited cutaneous systemic sclerosis
• diffuse cutaneous systemic sclerosis (dcSSc) also known as diffuse cutaneous systemic scleroderma, progressive cutaneous systemic scleroderma or progressive cutaneous systemic sclerosis
• systemic sclerosis sine scleroderma also known as limited systemic sclerosis
• systemic sclerosis overlap syndrome

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Signs & Symptoms

Systemic scleroderma can affect multiple organ systems and therefore lead to numerous symptoms and complications. The symptoms present, their severity, the rate of progression of the disease, the response to treatment and overall survival vary widely from person to person. Most people start to develop symptoms between the ages of 30 and 50. Symptoms related to the different organs involved are described below.

Skin

In most people, Raynaud phenomenon (RP) is the first manifestation of the disease. RP is characterized by blood vessel spams (vasospasm) in response to cold or stress. This most often occurs in the fingers and turns them white. Subsequently, as the vessels are occluded and cannot deliver oxygen, the fingers turn blue. This can also lead to severe pain and ulcers. After several minutes (usually 15 to 20, the vasospasms stop, and the fingers become red as blood flows again. Other skin symptoms include:

• Rapid growth of connective tissue in the skin (skin fibrosis) which first affects the fingers, hands and face and may be associated with tight and dry skin, itching (pruritus), fluid accumulation (edema) and increased or decreased skin pigmentation
• Telangiectasias, which are small, dilated blood vessels that can appear as red lesions visible through the skin
• Accumulation of calcium nodules in the fingers, forearm, elbows and knees, also known as calcinosis cutis that can lead to pain if the nodules are large

Nerves, muscles, and joints

Many people living with systemic scleroderma develop muscle, joint, or nerve disease. The symptoms may include:

• Muscle involvement can lead to muscle pain, weakness and muscle wasting (sarcopenia). Affected joints can be painful, stiff and swollen.
• Nerve involvement is less common but can lead to burning or tingling sensation or numbness.
  • Peripheral nerve problems (neuropathies) are less common than muscle problems in people with systemic sclerosis. However, some nerve-related conditions have been seen. These can include:
  • Numbness or tingling in the face (trigeminal sensory neuropathy)
  • Nerve problems that affect both movement and sensation (sensorimotor neuropathy)
  • Carpal tunnel syndrome which causes hand pain or numbness
  • Multiple nerve issues in different areas (called mononeuritis multiplex)
  • Lower back nerve root problems (lumbosacral radiculopathy)
  • Small-fiber neuropathy which can cause burning pain or temperature sensitivity
• • In some people the autonomic nervous system can be affected which is the part of the nervous system that is not under voluntarily control and is notably involved in self-regulation of the body and that can lead to symptoms such as dizziness when standing up (orthostasis), inappropriate heart rate response to exertion and inappropriate sweating.

Lungs

The two main pulmonary manifestations of systemic scleroderma are pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD). PAH and ILD are progressive conditions and can eventually become life-threatening. Taken together, they account for about half of all deaths related to systemic scleroderma.

• PAH is defined as increased pressure in the arteries of the lungs and can lead to shortness of breath (dyspnea), fatigue and failure of the right ventricle of the heart, which in turn can cause chest pain, fluid accumulation (edema) and transient loss of consciousness upon exertion (exertional syncope).
• ILD corresponds to scarring (fibrosis) of lung tissue, which prevents lung expansion and gas exchange. The two main symptoms of ILD are:
  • Progressive breathing difficulties (dyspnea)
  • Dry cough

Gastrointestinal tract

Nearly 90% of people with systemic sclerosis have some degree of gastrointestinal (GI) involvement and approximately one-half have moderate to severe symptoms.
Symptoms may include:

• Esophageal problems: The most commonly involved part of the gastrointestinal tract in systemic scleroderma is the esophagus:

• Heartburn due to acid reflux (a condition known as gastroesophageal reflux disease; GERD), choking on food and difficulty swallowing (dysphagia), which can be caused by the combination of an ineffective esophagus contraction (esophageal dysmotility) to allow food to reach the stomach and inability of the  lower esophageal sphincter (LES), which is a circular muscle between the esophagus and the stomach, to close properly

• Pain, bloating, bacterial growth, constipation, diarrhea and fecal incontinence due to fibrosis of the rest of the gastrointestinal tract which can prevent food from moving through properly (pseudo-obstruction)
• Gastrointestinal bleeding also occurs in some people due to dilation of veins in the stomach (gastric venous ectasias)
• Heart: Systemic scleroderma can affect many parts of the heart including the heart muscle itself (myocardium), the sac in which it is contained (pericardium), the arteries feeding it (coronary arteries and arterioles) and its electrical conduction system. The cardiovascular system is affected in about 10% to 30% of affected people and the manifestations vary depending on the affected part of the heart and can include:
• Chest pain
• Shortness of breath
• Hearth rhythm abnormalities (arrhythmias)
• Inability of the heart to pump blood forward optimally (heart failure)

Kidneys

The most common effects in systemic sclerosis include:

• Scleroderma renal crisis (SRC) is characterized by abrupt onset of severe high blood pressure (hypertension), renal failure and abnormal protein excretion in the urine (proteinuria). Microangiopathic hemolytic anemia (MAHA), a condition where the platelets in the blood excessively form clots in small vessels and when red blood cells travel in these vessels they get sheared by the platelet clots (hemolysis).
  • MAHA therefore leads to a decreased number of red blood cells (anemia) and platelets (thrombocytopenia).

Other

Liver and pancreas involvement is rare. However, hepatic disease due to primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) can occur particularly in people with the limited cutaneous form.

In addition to symptoms described above, people living with systemic scleroderma commonly have fatigue, anxiety, sexual dysfunction and concerns with body image. Although the disease is potentially fatal and is associated with an increased risk of mortality, many affected individuals can live long, productive lives.

Clinical subsets

Systemic scleroderma is typically divided into four subsets with different clusters of symptoms.

• Limited cutaneous systemic sclerosis (lcSSc) is associated with skin fibrosis limited to the hands, forearm, feet and face, and usually prominent telangiectasias and calcinosis cutis. People with lcSSc can develop pulmonary arterial hypertension but very rarely develop scleroderma renal crisis and interstitial lung disease.
• Diffuse cutaneous systemic sclerosis is associated with rapid and diffuse skin fibrosis and early occurrence of renal, cardiac and pulmonary complications.
• Systemic sclerosis sine scleroderma involves internal organ involvement of systemic scleroderma without the typical skin involvement.
• Systemic scleroderma overlap syndrome includes features of other connective tissue diseases such as rheumatoid arthritis or polymyositis and is notable for prominent joint and muscle involvement.

Distinguishing the clinical subset of systemic scleroderma in an affected person is important as it can help predict the progression of the disease and the associated complications. However, not all patients fall within a specific subset and overlap between different categories is common.

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Causes

The exact cause of systemic scleroderma is not fully understood. However, research suggests that a combination of genetic and environmental factors likely contributes to its development in people who are more susceptible.

It is thought to be an autoimmune disease, which means that it involves a dysregulated immune system that attacks the affected individual’s own body. The first event to occur is possibly injury to small blood vessels (microvascular injury). In healthy individuals, response to injury leads to recruitment of inflammatory mediators that facilitate repair. In individuals with systemic scleroderma, microvascular injury leads to disproportionate inflammatory mediator recruitment and excessive fibrous tissue deposition. This fibrous tissue can replace healthy tissue and lead to symptoms of systemic scleroderma such as skin thickening, internal organ scarring and associated complications.

It tends to occur more frequently in some families and often clusters with other autoimmune diseases. Scientific studies have identified specific genetic regions, particularly within the major histocompatibility complex (MHC), that are involved in the disease. This includes certain human leukocyte antigen (HLA) types such as HLA-DRB11104, DQA10501 and DQB1*0301. Other genes, not part of the HLA group, like PTPN22, NLRP1, STAT4, and IRF5, have also been linked to the risk of developing systemic sclerosis.

Certain environmental exposures are believed to trigger the disease in genetically predisposed individuals. These include infections such as cytomegalovirus (CMV), Epstein-Barr virus and parvovirus B19. Contact with substances like silica dust, organic solvents (including toluene and xylene), trichloroethylene and polyvinyl chloride have also been associated with increased risk. Importantly, smoking has not been shown to be a proven risk factor.

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Affected populations

Systemic sclerosis is a rare autoimmune disease that affects between 38 and 341 individuals per million worldwide (prevalence) and develops in 8 to 56 individuals per million each year (incidence). Rates vary based on geography and population. The disease is more common in the United States and Australia, and less common in Europe and Asia, including Japan. The highest known prevalence has been reported among Choctaw Native Americans in Oklahoma.

The disease most often starts to manifest in the fifth decade of life (age of onset). Although it most commonly occurs in females, males tend to have more severe disease. African American individuals tend to have a younger age of onset, higher rates of the diffuse cutaneous subtype and overall, more severe disease.

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Disorders with Similar Symptoms

Symptoms of the following disorders may be similar to those of systemic scleroderma. Comparisons may be useful for a differential diagnosis:

Localized scleroderma (also known as morphea) is characterized by skin symptoms similar to those seen in systemic scleroderma but without internal organ involvement. It is considered a disease distinct from systemic scleroderma.

Polymyositis is a type of inflammatory muscle disease (myopathy) characterized by inflammatory and degenerative changes in the muscles, leading to symmetric weakness and some degree of muscle wasting (atrophy). The areas principally affected include the muscles closest to and within the trunk of the body such as the hip, shoulders, arms, pharynx and neck. Polymyositis occurs most often in females over 20 years of age, but males can also be affected. Muscle weakness usually happens over days, weeks or months. Some affected people have muscle pain, breathing problems and trouble swallowing.

Dermatomyositis is characterized by muscle involvement similar to what is seen in polymyositis but also involves the skin. Associated skin abnormalities often include a distinctive reddish-purple rash (heliotrope rash) on the upper eyelid or across the cheeks and bridge of the nose in a “butterfly” distribution and on the forehead and scalp. Other characteristic rashes include scaling and redness of the knuckles, elbows, knees and/or other extensor regions (Gottron papules and sign); an abnormal accumulation of fluid (edema) in body tissues surrounding the eyes and/or other features.

Systemic lupus erythematosus (SLE; lupus) is a chronic autoimmune disease affecting multiple organ systems. The symptoms are variable depending on the affected individual and can overlap with those seen in systemic scleroderma. Common disease manifestations include fatigue, joint pain (arthralgia), muscle pain (myalgia), facial (butterfly) rash and kidney disease. The gastrointestinal tract, lungs, heart and central nervous system can also be affected.

Mixed connective tissue disease (MTCD) is an uncommon systemic inflammatory rheumatic disease. MCTD is a specific subset of the broader category of rheumatic “overlap syndromes”, a term used to describe when a patient has features of more than one classic inflammatory rheumatic disease. These classic rheumatic diseases include systemic lupus erythematosus, polymyositis, systemic scleroderma and rheumatoid arthritis. Common symptoms seen in affected individuals include Raynaud phenomenon, joint pain (arthralgia) or inflammation (arthritis), muscle weakness and inflammation (myositis) and involvement of internal organs such as the gastrointestinal tract, lung, heart and kidneys.

Amyloidosis is a group of disorders characterized by accumulation of abnormally folded protein (amyloid) that can occur throughout the body. It can occur as a disorder of its own or in the context of another predisposing disorder. Amyloid deposition in the skin can lead to swelling and easy bruising. Joint and muscle pain can also result from amyloid deposition. As with systemic scleroderma, internal organ involvement can occur and can notably lead to renal, cardiac, pulmonary and nervous system disease.

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Diagnosis

Systemic scleroderma is a complex disorder that can be difficult to diagnose. In most people, the diagnosis starts with a complete medical history and physical examination. If systemic scleroderma is suspected, lab tests can be ordered.

Notably, certain antibodies reacting against components of an individual’s body (autoantibodies) can be identified in systemic scleroderma. Antinuclear antibodies are present in approximately 95% of individuals. However, they are also present in other autoimmune conditions and in healthy individuals. Antitopoisomerase I (anti-Scl-70) antibodies and anti-RNA polymerase III antibodies are associated with dcSSc, while anticentromere antibodies can be seen in lcSSc. If antibodies titers are indicative of systemic scleroderma, specific tests will be performed to confirm the diagnosis and evaluate for internal organ involvement.

Pulmonary function tests (PFTs) are breathing tests used to assess the lungs capacity to move air and diffuse it to the blood. They are often complemented by a computed tomography (CT) scan of the chest to visually assess the structure of the lungs.

The heart and pulmonary arteries can be imaged with cardiac echocardiography, which uses ultrasound waves to reconstruct and visualize anatomical structures.

Renal function can notably be evaluated by measuring creatinine levels in the blood and by urine analysis.

Further testing might be indicated depending on the symptoms and characteristics of the affected individual.

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Standard Therapies

Treatment and Management

Although there is currently no cure for systemic scleroderma, treatment focuses on managing symptoms, slowing disease progression and monitoring for complications. Most people require long-term, coordinated care involving several specialists. As systemic scleroderma is an autoimmune condition, medications that suppress the immune system, known as immunosuppressants, are often prescribed. These may be particularly important for patients with extensive skin thickening, lung inflammation, myocarditis (inflammation of the heart muscle), or severe muscle and joint inflammation. Common immunosuppressants include methotrexate, mycophenolate mofetil (MMF), azathioprine and cyclophosphamide. In some people, stem cell transplantation may be considered. Glucocorticoids like prednisone are sometimes used, although their long-term use is limited due to potential side effects and increased risk of kidney complications.

Skin, muscles, joints, and nerves
For people with extensive skin thickening or inflammatory involvement of muscles and joints, immunosuppressants can be helpful. Raynaud phenomenon, a common symptom in scleroderma, is usually treated with calcium channel blockers such as amlodipine (Norvasc) or nifedipine (Procardia XL). Calcinosis cutis, a condition involving calcium deposits under the skin, may be treated with bisphosphonates or surgical removal for larger deposits. Skin pigment changes and visible small blood vessels (telangiectasias) can be improved with laser therapy and concealed with cosmetics. Rheumatologists typically oversee these symptoms, with dermatologists involved for skin-related concerns.

Neurologists may be involved when nerve-related symptoms arise. For example, medications such as pregabalin (Lyrica) or gabapentin (Neurontin) can help manage neuropathic pain caused by nerve damage.

Lungs
Early diagnosis and management of lung complications, particularly interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), are crucial. ILD is traditionally treated with immunosuppressants, and since 2019, nintedanib (Ofev) has been approved to slow lung function decline in patients with scleroderma-related ILD. PAH is treated with medications such as epoprostenol (Flolan) or bosentan (Tracleer), which help reduce blood pressure in the lungs. Pneumologists typically manage these issues, and in severe cases, lung transplantation may be considered by surgeons specializing in lung problems.

Gastrointestinal tract
Most patients with systemic scleroderma are treated with medication such as pantoprazole (Protonix), that decreases acid production by the stomach. Some patients are also prescribed medication that increases gastrointestinal motility such as metoclopramide (Reglan) or domperidone (Motilium). Antibiotics can be used in patients with gastrointestinal bacterial overgrowth and associated symptoms. Gastroenterologists will often be involved, especially if other gastrointestinal manifestations that require more specific treatments are present.

Heart
The treatment of systemic scleroderma-associated cardiac disease will depend on the specific cardiac manifestation that is present. Heart failure might require a specific kind of medication known as ACE inhibitors (for instance, perindopril), that is used for high blood pressure and medications that make the patient urinate (diuretics) such as furosemide (Lasix) to decrease fluid overload. As described above, immunosuppressants can be used in individuals with myocarditis. Cardiologists follow systemic scleroderma patients to manage potential cardiac complications.

Recommendations include annual screening with echocardiogram, electrocardiogram (ECG), pulmonary function tests, NT-proBNP (if >3 years since diagnosis or low DLCO) and frequent blood pressure checks (daily or 2x/week) in high-risk people.

Kidneys
In addition to supportive management, ACE inhibitors are the mainstay of treatment for scleroderma renal crisis (SRC). Of note, chronic high doses of glucocorticoids are associated with an increased risk of SRC, which is another reason why their use is limited as much as possible in systemic scleroderma. In severe cases of SRC, dialysis and even renal transplantation might be required if renal function does not improve after the crisis. Nephrologists are physicians specializing in the diagnosis and management of kidney (renal) diseases.

Other
Depending on the affected individual and their needs, other medical professionals will be involved such as nurses, social workers, physiotherapists, occupational therapists and psychologists.

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Clinical Trials and Studies

As the mechanisms responsible for the development and progression of the disease are uncovered, new medications or medications already on the market for other diseases are tested in clinical trials. Examples of targeted therapies being investigated include rilonacept and tocilizumab, which respectively target IL-1 and IL-6, two inflammatory mediators thought to be important in systemic scleroderma

Newer therapies are being studied that target the root causes of the disease rather than just the symptoms. Janus kinase (JAK) inhibitors are one such example. These medications act on immune pathways that drive inflammation and fibrosis in scleroderma. Early research suggests they may help reduce skin and organ damage, though more studies are needed to confirm their long-term safety and effectiveness.

UV-A1 phototherapy is emerging as a promising non-invasive treatment option for improving skin fibrosis and modulating immune activity. While still under investigation, initial results suggest it may improve skin elasticity and reduce inflammation, offering potential benefit alongside standard therapies.

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/

For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

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References

INTERNET

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Denton CP. Pathogenesis of systemic sclerosis (scleroderma). UpToDate. Last updated: Aug 02, 2024. https://www.uptodate.com/contents/pathogenesis-of-systemic-sclerosis-scleroderma Accessed June 18, 2025.

Kaye-Barrett SA, Denton CP. Gastrointestinal manifestations of systemic sclerosis (scleroderma). UpToDate. Last updated: Jan 16,2025.
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King TE. Approach to the adult with interstitial lung disease: Clinical evaluation. UpToDate. Last updated: Jun 28, 2024.  https://www.uptodate.com/contents/approach-to-the-adult-with-interstitial-lung-disease-clinical-evaluation Accessed June 18, 2025.

Mukherjee M, Shah S, Varga J. Cardiac manifestations of systemic sclerosis (scleroderma). UpToDate. Last updated: Dec 01, 2023. https://www.uptodate.com/contents/cardiac-manifestations-of-systemic-sclerosis-scleroderma Accessed June 18, 2025.

Paik JJ, Varga J. Neuromuscular manifestations of systemic sclerosis (scleroderma). UpToDate. Last updated: Jan 18, 2025.
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Varga J. Overview of pulmonary complications of systemic sclerosis (scleroderma). UpToDate. Last updated: Jan 02, 2024. https://www.uptodate.com/contents/overview-of-pulmonary-complications-of-systemic-sclerosis-scleroderma Accessed June 18, 2025.

Varga J, Fenves AZ. Renal disease in systemic sclerosis (scleroderma), including scleroderma renal crisis. UpToDate. Last updated: Oct 14, 2024. https://www.uptodate.com/contents/renal-disease-in-systemic-sclerosis-scleroderma-including-scleroderma-renal-crisis Accessed June 18, 2025.

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JOURNAL ARTICLES

Nagy S, Tehrani L, Kesselman MM. Effect of UV-A1 Phototherapy Treatment on Scleroderma: A Systematic Review. Cureus. 2025;17(4):e82899. Published 2025 Apr 24. doi:10.7759/cureus.82899

Sener S, Sener YZ, Batu ED, Sari A, Akdogan A. A systematic literature review of Janus kinase inhibitors for the treatment of systemic sclerosis. J Scleroderma Relat Disord. Published online May 22, 2025. doi:10.1177/23971983251342697

Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699. doi:10.1016/S0140-6736(17)30933-9

Allanore Y, Simms R, Distler O, et al. Systemic sclerosis. Nat Rev Dis Primers. 2015;1:15002. Published 2015 Apr 23. doi:10.1038/nrdp.2015.2.

Barnes J, Mayes MD. Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol. 2012;24(2):165-170. doi:10.1097/BOR.0b013e32834ff2e8

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