SYSTEMIC SCLEROSIS

Systemic sclerosis is a rare autoimmune disease with a variable course that can affect the skin, blood vessels, and certain internal organs. Symptoms differ greatly from one patient to another: some people mainly show Raynaud's phenomenon and skin changes, while others may develop lung, digestive, cardiac, or kidney involvement.

There is no single treatment for systemic sclerosis. The medical plan is built based on the form of the disease, symptoms, antibodies, investigation results, and the risk of organ involvement. Early diagnosis, periodic monitoring, and collaboration between specialties allow complications to be identified quickly and the treatment to be adjusted.

What is systemic sclerosis?

Systemic sclerosis, also called systemic scleroderma, is an autoimmune rheumatologic disease characterized by three main mechanisms:

• abnormal functioning of the immune system;
• damage to small blood vessels;
• excessive collagen production and the development of fibrosis.

Fibrosis can cause the skin to thicken and stiffen, but it can also affect the lungs, the digestive tract, the heart, or the kidneys. The disease should not be confused with localized scleroderma, which mainly affects the skin and does not have the same internal involvement profile.

There are two main clinical forms:

• limited cutaneous systemic sclerosis, in which skin changes are mainly located on the face, hands, forearms, and the segments below the elbows or knees;
• diffuse cutaneous systemic sclerosis, in which skin thickening can extend proximally and may be associated with a higher risk of early internal organ involvement.

The term progressive systemic sclerosis is sometimes used to describe the evolving nature of the disease, but the pace of progression is not the same for every patient. Some forms progress slowly, while others require more intensive investigation and treatment from the early years.

Among the most well-known symptoms of systemic sclerosis are:

• fingers that turn white, blue, or red in the cold;
• swollen fingers;
• thickening and tightening of the skin;
• joint pain and stiffness;
• gastroesophageal reflux and difficulty swallowing;
• difficulty breathing or reduced exercise tolerance;
• ulcers on the fingers;
• persistent fatigue.

Both the limited and diffuse forms can affect internal organs. The difference between them mainly concerns the extent of the skin changes, not the absence or presence of systemic involvement.

How is the diagnosis made?

The diagnosis of systemic sclerosis is not based on a single test. The doctor correlates the symptoms, the clinical examination, capillaroscopy, the autoantibody profile, and the investigations used to assess the organs.

Rheumatology consultation

The rheumatology consultation is the first step when systemic sclerosis is suspected. The doctor discusses the onset of symptoms, episodes of Raynaud's phenomenon, skin changes, joint pain, digestive symptoms, and any breathing difficulties.

The clinical examination may look at:

• the degree of skin thickening;
• the mobility of the fingers and joints;
• the presence of digital ulcers;
• telangiectasias;
• calcium deposits in the tissues;
• hand swelling;
• signs of vascular involvement;
• exercise tolerance.

Raynaud's syndrome is often one of the first symptoms. However, most people with Raynaud's phenomenon do not have systemic sclerosis. Suspicion increases when it is associated with swollen fingers, changes in the nailfold capillaries, ulcers, or specific autoantibodies.

Blood tests (specific antibodies)

Blood tests can reveal antinuclear antibodies and autoantibodies associated with systemic sclerosis.

Among the most important are:

• anticentromere antibodies;
• anti-topoisomerase I antibodies, also known as anti-Scl-70;
• anti-RNA polymerase III antibodies.

These antibodies support the diagnosis and can provide information about certain clinical risks. For example, anti-Scl-70 is more frequently associated with interstitial lung involvement, while anti-RNA polymerase III antibodies may be associated with a higher risk of scleroderma renal crisis.

A positive antibody result is not sufficient on its own to establish the diagnosis. Interpretation must be done in the context of the symptoms, the clinical examination, and the other investigations. Specific autoantibodies, capillaroscopy, and clinical features are all integrated into the patient's overall evaluation.

Capillaroscopy

Capillaroscopy is a non-invasive investigation that examines the small vessels at the base of the nails. The doctor uses a magnifying device to analyze the shape, size, and distribution of the capillaries.

In systemic sclerosis, the following may appear:

• dilated capillaries;
• micro-hemorrhages;
• a reduced number of capillaries;
• areas with no visible vessels;
• vessels with an abnormal architecture.

The investigation is especially important for people with Raynaud's phenomenon. A normal result may point toward primary Raynaud's phenomenon, while a specific pattern can support the suspicion of a connective tissue disease.

Capillaroscopy is not painful, does not use radiation, and can be repeated for monitoring.

Imaging and multidisciplinary evaluation

After the suspicion is raised or the diagnosis is confirmed, the doctor evaluates possible organ involvement. The investigations are chosen individually and may include:

• pulmonary function tests;
• high-resolution chest CT scan;
• echocardiography;
• electrocardiogram;
• cardiac MRI, in selected cases;
• right heart catheterization, when pulmonary hypertension is suspected;
• kidney function tests;
• regular blood pressure monitoring;
• endoscopy, manometry, or other digestive tests;
• musculoskeletal ultrasound or joint MRI, if locomotor symptoms are present.

Imaging and functional tests are not automatically performed in the same way for every patient. They are recommended based on symptoms, the disease subtype, autoantibodies, and individual risk.

Treatment options

Treatment for systemic sclerosis is directed at the disease's specific manifestations. There is currently no medication that fully cures the condition or reverses all fibrotic changes. However, there are therapies that can control inflammation, reduce symptoms, and slow the progression of certain complications.

Medication (immunosuppressants, vasodilators)

Immunosuppressive medications may be recommended when there is progressive skin involvement, inflammation, or interstitial lung involvement.

Vasodilators are used for vascular involvement. For Raynaud's phenomenon, the doctor may recommend calcium channel blockers. In more severe cases or in the presence of digital ulcers, other classes may be used, such as phosphodiesterase type 5 inhibitors or intravenous prostanoids.

Specific treatments exist for pulmonary arterial hypertension, chosen and monitored by a team experienced in this complication. Medication selection depends on the cardiopulmonary evaluation and the severity of the disease.

Symptomatic treatment

Symptomatic treatment is adapted to the affected area.

For reflux and digestive involvement, the doctor may recommend:

• proton pump inhibitors;
• medications that support digestive motility;
• adjusting meals;
• treating constipation or diarrhea;
• gastroenterological evaluation.

For musculoskeletal symptoms, the following may be considered:

• analgesics;
• anti-inflammatory treatment, if indicated;
• physiotherapy;
• mobility exercises;
• orthoses;
• management of contractures.

Digital ulcers require local protection, treatment of infection if it occurs, and control of vascular involvement. Wounds that do not heal, become very painful, or change color must be evaluated promptly.

Scleroderma renal crisis is a medical emergency. It can be associated with a sudden rise in blood pressure and deterioration of kidney function. Treatment with angiotensin-converting enzyme inhibitors must be started quickly, under medical supervision.

Long-term monitoring

Monitoring is an essential component of systemic sclerosis treatment. Some complications may initially progress without obvious symptoms.

The follow-up plan may include:

The frequency of investigations is adapted to the form of the disease, its duration, symptoms, and previous results.

Recovery and long-term management

Medical recovery does not treat the autoimmune mechanism, but it can help maintain mobility, reduce functional limitation, and support the continuation of daily activities.

Physiotherapy and maintaining mobility

Physiotherapy may be recommended for:

• maintaining finger mobility;
• preventing or limiting contractures;
• improving joint mobility;
• maintaining muscle strength;
• posture re-education;
• supporting exercise tolerance;
• maintaining mouth opening in the presence of microstomia.

The program must be adapted to the level of involvement. Aggressive exercises or forcing the joints can increase pain and tissue irritation.

Exercises may include:

• gentle mobilization of the hands and fingers;
• joint extension exercises;
• controlled stretching;
• breathing exercises;
• aerobic activity at a tolerated intensity;
• functional exercises for daily activities.

Consistency matters more than high intensity. The physical therapist adjusts the program if ulcers, pain, marked fatigue, or cardiopulmonary involvement occur.

Managing pain and stiffness

Pain can have different causes: joint inflammation, contractures, tendon involvement, skin changes, or poor circulation.

The plan may include:

• controlled exercises;
• mobilization techniques;
• ergonomic recommendations;
• physiotherapy, if indicated;
• medication established by the doctor;
• adapting activities.

The use of heat should be discussed with the specialist, especially if there are sensory disturbances, ulcers, or vascular involvement. Massage can be used selectively and gently, without pressure on areas with fragile skin, calcinosis, or lesions.

Lifestyle and self-care

Self-care measures complement medical treatment.

It is recommended to:

• avoid smoking;
• protect your hands and feet from the cold;
• use gloves and appropriate clothing;
• avoid sudden temperature changes;
• moisturize your skin with recommended products;
• avoid injuries to the fingers;
• maintain an adapted level of physical activity;
• follow the treatment for reflux;
• monitor your blood pressure as instructed;
• report new symptoms.

Quitting smoking is especially important because nicotine causes vasoconstriction and can worsen Raynaud's phenomenon and digital ulcers.

The multidisciplinary approach at Centrokinetic

Systemic sclerosis can affect multiple organs and functions. This is why a coordinated evaluation reduces the risk of certain manifestations being assessed in isolation.

Collaboration between rheumatology, imaging, recovery, and neurology

The rheumatologist coordinates the disease evaluation and determines the need for additional consultations. Depending on symptoms, the patient may benefit from collaboration between:

• rheumatologist;
• rehabilitation physician;
• physical therapist;
• radiologist;
• neurologist;
• orthopedic surgeon;
• cardiologist;
• pulmonologist;
• gastroenterologist;
• nutritionist.

The multidisciplinary approach is important because systemic sclerosis is a complex disease, with vascular, cutaneous, musculoskeletal, and visceral manifestations.

Investigations available at the clinic

Depending on the medical indication, the evaluation can be completed with:

• musculoskeletal ultrasound;
• MRI of the joints;
• electromyography;
• functional evaluations;
• medical tests;
• interdisciplinary consultations.

Investigations performed at the clinic do not replace the necessary cardiology, pulmonology, kidney, or digestive evaluations. The rheumatologist determines which tests are relevant and where they should be performed.

Personalized treatment plan

The personalized plan takes into account:

• the limited or diffuse form;
• the duration of the disease;
• the predominant symptoms;
• the antibodies identified;
• organ involvement;
• age;
• activity level;
• exercise tolerance;
• previous treatments.

Goals may include controlling vascular symptoms, protecting lung function, maintaining mobility, managing reflux, and preventing contractures. The program is periodically reassessed and adapted to progress.

When should you see a rheumatologist?

A consultation is recommended when symptoms suggest an autoimmune or vascular disease, especially if several manifestations appear at the same time.

Symptoms that require prompt evaluation

Schedule a consultation if you notice:

• recently developed Raynaud's phenomenon;
• persistently swollen fingers;
• thickening or tightening of the skin;
• ulcers on the fingers;
• stiffness and reduced hand mobility;
• persistent reflux or difficulty swallowing;
• difficulty breathing;
• reduced exercise tolerance;
• palpitations;
• an unexplained rise in blood pressure.

Seek urgent medical attention if the following occur:

• severe difficulty breathing;
• chest pain;
• a sudden rise in blood pressure;
• a decrease in urine output;
• confusion;
• very painful, cold fingers, or fingers with persistent color changes.

The importance of early diagnosis

Early diagnosis allows organs to be evaluated before involvement becomes advanced. It also helps establish the risk profile and choose the appropriate treatment.

Early intervention can help with:

• controlling symptoms;
• reducing the risk of certain complications;
• slowing the progression of certain manifestations;
• maintaining mobility;
• planning monitoring.

Regarding systemic sclerosis and life expectancy, the prognosis varies considerably. The form of the disease, lung involvement, pulmonary hypertension, cardiac involvement, and kidney involvement all influence the course. Advances in monitoring and treatment have improved the prognosis, but each case must be evaluated individually.

Schedule a consultation

If you notice symptoms of systemic sclerosis or have already received this diagnosis, a complete rheumatological evaluation helps you understand which investigations and treatments are appropriate.

At Centrokinetic, the evaluation is adapted to your symptoms and medical history. The team can integrate the rheumatology consultation with the functional evaluation, the available investigations, and the recovery program.

Schedule a consultation at Centrokinetic for the evaluation of systemic sclerosis and to establish a personalized medical plan.

Frequently asked questions about systemic sclerosis

Can systemic sclerosis be cured?

Currently, there is no curative treatment. However, there are therapies that can keep symptoms under control, treat organ involvement, and slow the progression of certain manifestations.

What is the difference between limited and diffuse systemic sclerosis?

The difference is mainly based on the extent of skin involvement. Both forms can involve internal organs and require monitoring.

Does progressive systemic sclerosis progress the same way in all patients?

No. The pace and type of involvement vary. Some patients have a slow course, while others require more intensive treatment and frequent investigations.

Can physiotherapy stop the disease?

No. Physiotherapy does not change the autoimmune mechanism, but it can support mobility, joint function, posture, and the ability to carry out daily activities.

What tests confirm the diagnosis?

There is no single test that confirms all cases. The diagnosis is based on symptoms, the rheumatology consultation, autoantibodies, capillaroscopy, and organ evaluation.

What influences life expectancy in systemic sclerosis?

The prognosis depends on the form of the disease, the speed of progression, lung, cardiac, or kidney involvement, and the response to treatment. Early evaluation and periodic monitoring are important.

Disclaimer: This material is for informational purposes only and does not replace a specialist medical consultation. The diagnosis, investigations, medication treatment, and recovery program must be established individually by the medical team, based on the form of the disease, symptoms, and organ involvement.

Information sources

• Del Galdo, Francesco, et al. "EULAR Recommendations for the Treatment of Systemic Sclerosis: 2023 Update." Annals of the Rheumatic Diseases, vol. 84, no. 1, 2025, pp. 29-40, doi:10.1136/ard-2024-226430. https://doi.org/10.1136/ard-2024-226430. Accessed Aug. 5, 2026.
• National Institute of Arthritis and Musculoskeletal and Skin Diseases. "Scleroderma: Diagnosis, Treatment, and Steps to Take." NIAMS, Sept. 2023, https://www.niams.nih.gov/health-topics/scleroderma/diagnosis-treatment-and-steps-to-take. Accessed Aug. 5, 2026.
• Mayo Clinic. "Scleroderma - Symptoms and Causes." Mayo Clinic, https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952. Accessed Aug. 5, 2026.