Scleroderma and the Heart: When Is Further Evaluation Needed?

Skin that feels increasingly stiff, fingers that are often cold or change color, and shortness of breath that appears during activity can leave a person wondering: is this a sign of scleroderma? Concerns like this are understandable, especially because scleroderma is not a widely known condition, yet it can have effects that go beyond changes in the skin.

Scleroderma is an autoimmune disease that can cause inflammation and thickening of tissue, especially the skin. In some people, the condition is limited to the skin. However, in the systemic type, or systemic sclerosis, the disease can involve the blood vessels and internal organs, including the lungs, heart, kidneys, and digestive tract.

Put simply, scleroderma is not only about hardened skin. In the systemic type, symptoms such as shortness of breath, tiring easily, palpitations, chest pain, or leg swelling need attention because they may be related to the lungs, the pulmonary blood vessels, or the heart. The right tests help the doctor understand the condition as a whole, so patients do not have to guess on their own.

This article discusses what scleroderma is, the symptoms to recognize, its causes and risk factors, the link between scleroderma and the heart and lungs, the tests that may be needed, treatment options, and when it is advisable to consult a doctor.

01 / Autoimmune Disease

What Is Scleroderma?

Scleroderma is an autoimmune disease that causes body tissue to become thicker, stiffer, or tighter. The condition is most often recognized by changes in the skin, but in its systemic form, scleroderma can also affect the blood vessels and internal organs.

A Brief Definition of Scleroderma

The term “scleroderma” is often associated with “hard skin”. In medical practice, however, the disease does not always stop at the skin. In some patients, the autoimmune process can also affect the blood vessels, digestive tract, lungs, heart, kidneys, muscles, or joints.

The term systemic sclerosis, or SSc, is usually used for the systemic form of scleroderma. This is the form that is more relevant when discussing the possible involvement of internal organs.

In scleroderma, the body can produce too much collagen. Collagen is actually an important protein that forms connective tissue. However, when too much of it is produced and it builds up in tissues, the skin or certain organs can become stiffer or thicker, or stop working properly.

Why Is Scleroderma an Autoimmune Disease?

Scleroderma is classified as an autoimmune disease because the immune system reacts inappropriately against the body’s own tissues. This reaction can trigger inflammation, changes in the small blood vessels, and a buildup of connective tissue.

Why this process begins has not been traced to a single cause. Experts believe scleroderma is likely influenced by a combination of immune, genetic, hormonal, and environmental factors. It is therefore not accurate to see scleroderma as a disease caused by one particular habit or by a patient’s “mistake”.

Keep in mind that symptoms resembling scleroderma are not enough for a self-diagnosis. An examination by a doctor is still needed to determine whether the complaints are truly related to scleroderma or to another condition that resembles it.

02 / Autoimmune Disease

Types of Scleroderma

Scleroderma is divided into two major groups: localized scleroderma and systemic scleroderma. This distinction is important because each has a different pattern of symptoms and different monitoring needs.

Localized Scleroderma

Localized scleroderma is usually limited to the skin and the tissue just beneath it. This form can appear as areas of skin that are thickened, discolored, or harder, or that form lines on certain parts of the body. The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) explains that localized scleroderma affects the skin and the structures directly beneath it.

In localized scleroderma, internal organ involvement is generally not a main feature. Even so, skin changes still need to be assessed by a doctor, especially if the affected area spreads, interferes with movement, or occurs on the face and limbs.

Systemic Scleroderma

Systemic scleroderma, or systemic sclerosis, is the form that can involve many body systems. Besides the skin, the condition can affect the blood vessels, lungs, heart, kidneys, and digestive tract.

In the context of heart health, the systemic type is important because it can be linked with cardiopulmonary complaints, meaning complaints involving the lungs, the pulmonary blood vessels, and the heart. For example, a person with systemic scleroderma who experiences shortness of breath, reduced exercise tolerance, palpitations, or leg swelling should be evaluated more thoroughly.

Limited and Diffuse Scleroderma

Systemic scleroderma is often divided into two forms based on the extent of skin involvement: limited and diffuse.

In the limited form, skin changes are usually more restricted, for example to the fingers, hands, face, forearms, or lower legs. In the diffuse form, skin changes can be more widespread and develop more quickly. The National Institute of Arthritis and Musculoskeletal and Skin Diseases explains that the limited and diffuse classification describes how extensive the skin involvement is in systemic scleroderma.

This classification is not meant to make patients anxious. Instead, it helps the doctor assess the risk of organ involvement, determine the appropriate tests, and plan long-term monitoring.

03 / Autoimmune Disease

Symptoms of Scleroderma

The symptoms of scleroderma can differ from person to person. Some people first notice changes in their skin. Others seek care because their fingers often change color in the cold, or because they have difficulty swallowing, tire easily, or feel short of breath during activity.

Symptoms in the Skin and Fingers

Skin symptoms are among the easiest signs to recognize. Complaints that may appear include:

• skin that feels tight or thickened;
• skin that looks shiny because it is stretched;
• swollen or stiff fingers;
• fingers that are hard to move;
• skin on the hands, face, feet, or other areas that feels harder;
• color changes in certain areas of the skin.

Hardening and tightening of the skin are very common symptoms of scleroderma. The areas affected first usually include the fingers, hands, feet, and face.

However, not all dry, stiff, or tight skin means scleroderma. Other skin conditions, circulation problems, medication side effects, or other autoimmune diseases can also cause similar complaints. That is why the pattern of symptoms and a doctor’s examination are important.

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• Often-Overlooked Lupus Symptoms and When to Get an ANA Test
• What Is Rheumatoid Arthritis? Recognize the Symptoms, Causes, and Link to the Heart

Raynaud’s Phenomenon, or Fingers Changing Color

One sign often associated with scleroderma is Raynaud’s phenomenon. This occurs when the small blood vessels in the fingers or toes overreact to cold or emotional stress.

During a Raynaud’s episode, the fingers can feel numb or painful, or turn white, bluish, or grayish, then reddish as blood flow returns. However, Raynaud’s can also occur in people who do not have scleroderma.

So when should you get checked? If your fingers often change color, feel painful, develop sores at the fingertips, or the skin on your fingers becomes increasingly tight, it is advisable to consult a doctor. An examination helps distinguish ordinary Raynaud’s from Raynaud’s related to a systemic autoimmune disease.

Read Also:

• Be Alert to Vasculitis: Symptoms, Risks, and Prevention

Symptoms in the Digestive Tract

Systemic scleroderma can also affect the digestive tract. Complaints that may appear include:

• a burning feeling in the chest, or heartburn;
• acid reflux;
• difficulty swallowing;
• bloating;
• diarrhea or constipation;
• feeling full quickly after eating;
• problems absorbing food in certain cases.

Scleroderma can affect the digestive tract from the esophagus to the rectum. As a result, its symptoms can vary from heartburn to changes in bowel habits.

Even so, not all GERD or bloating means scleroderma. However, if a person has already been diagnosed with scleroderma and has persistent digestive complaints, the doctor can assess whether these complaints are related to impaired movement of the digestive tract.

Read Also:

• Burning Chest and Shortness of Breath: GERD or a Heart Problem?

Symptoms in the Lungs and Heart

In some patients, systemic scleroderma can involve the lungs, the pulmonary blood vessels, or the heart. Symptoms to watch for include:

• shortness of breath during activity;
• tiring quickly or a reduced ability to be active;
• a persistent dry cough;
• chest discomfort;
• palpitations;
• dizziness or nearly fainting;
• swelling in the feet or ankles.

When scleroderma affects the heart or lungs, complaints can include shortness of breath, reduced exercise tolerance, dizziness, pulmonary hypertension, heart rhythm disorders, or heart failure in some people.

These complaints are not always caused by scleroderma. However, in patients with scleroderma or suspected systemic scleroderma, heart and lung symptoms should not be dismissed as “just tiredness” if they persist, get worse, or interfere with daily activities.

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• Irregular Heartbeat? Recognize Arrhythmia Before It Is Too Late!
• Heart Failure: A Hidden Threat You Need to Recognize and Watch Out For
• Just 30 Minutes! Learn About Your Heart Condition With Echocardiography

04 / Autoimmune Disease

Causes and Risk Factors

The cause of scleroderma is not yet known for certain. However, research shows that autoimmune processes, changes in the blood vessels, genetic factors, and possible environmental triggers may play a role.

Causes of Scleroderma

In scleroderma, the immune system can trigger connective tissue cells to produce excess collagen. As a result, collagen builds up in the skin or certain organs and makes the tissue stiffer or thicker.

Experts do not yet know exactly what starts this process. However, a combination of immune system problems, genetics, and environmental triggers likely plays a role.

This point is important: scleroderma is not a disease that appears because someone “did not take good care of themselves”. A healthy lifestyle is still important for supporting your general health, but scleroderma itself is a complex autoimmune condition that requires medical evaluation.

Factors That May Increase Risk

Several factors may be linked with a higher likelihood of scleroderma, including:

• female sex;
• adulthood, especially between the ages of 30 and 50;
• a family history or certain genetic factors;
• other autoimmune conditions;
• possible exposure to certain environmental factors in some cases.

Scleroderma is more common in women and usually appears between the ages of 30 and 50. Genetic factors, environmental triggers, and immune conditions can affect risk, although a specific trigger is not always found in every person.

Risk factors are not the same as a definite cause. A person can have risk factors and never develop scleroderma. Conversely, a person without any clear risk factors can still develop the condition.

05 / Autoimmune Disease

Effects of Scleroderma on the Heart and Lungs

In systemic scleroderma, the blood vessels, lungs, and heart can also be affected. That is why organ monitoring is an important part of a comprehensive approach.

Effects on the Blood Vessels

Systemic scleroderma can affect the small blood vessels. This is one reason why Raynaud’s phenomenon, fingertip sores, or impaired peripheral blood flow often come up in discussions of this disease.

In the recommendations of the Indonesian Rheumatology Association (IRA), systemic sclerosis is described as a systemic autoimmune rheumatic disease characterized by immune dysregulation, fibrosis of the skin and visceral organs, and vasculopathy. The guideline also names Raynaud’s phenomenon, digital ulcers, and pulmonary arterial hypertension as features of the microvascular abnormalities in SSc.

For lay readers, the main message is simple: if scleroderma is systemic, the doctor does not only look at the skin. The doctor also assesses whether the blood vessels and other organs are affected.

Scleroderma and the Lungs

The lungs are one of the important organs to watch in systemic scleroderma. Two conditions that are often discussed are interstitial lung disease and pulmonary hypertension.

Interstitial lung disease occurs when lung tissue becomes inflamed or scarred. This condition can reduce lung function and cause shortness of breath, a dry cough, or tiring quickly.

The Indonesian Rheumatology Association guideline states that every patient with SSc should be evaluated for lung involvement from the time of diagnosis. The same guideline also notes that many patients with interstitial lung disease in SSc show no symptoms, especially in the early phase.

So patients do not need to wait until they are severely short of breath to discuss lung monitoring with their doctor. In the systemic condition, regular tests can help detect changes earlier.

Scleroderma and the Heart

The heart can be affected directly or indirectly in systemic scleroderma. Directly, the condition can be related to heart muscle problems, rhythm disorders, inflammation, or changes in the structure and function of the heart. Indirectly, the heart can be put under strain if pulmonary hypertension or interstitial lung disease develops.

The Indonesian Rheumatology Association guideline states that every patient with SSc should be evaluated for heart involvement. Heart involvement can be primary, caused by the SSc process itself, or secondary to pulmonary arterial hypertension, interstitial lung disease, or kidney disease.

Signs to watch for include:

• recurring palpitations;
• chest pain or chest discomfort;
• shortness of breath during activity;
• dizziness or fainting;
• unusual fatigue;
• swelling in the feet.

These symptoms do not automatically mean the heart is already affected. However, if they appear in a patient with scleroderma, a heart evaluation can help the doctor assess whether the complaints are related to heart rhythm, heart function, pressure in the pulmonary blood vessels, or another cause.

Pulmonary Hypertension in Scleroderma

Pulmonary hypertension is increased pressure in the blood vessels of the lungs. When this pressure rises, the right side of the heart has to work harder to pump blood to the lungs.

The NHLBI explains that connective tissue disorders such as scleroderma are among the conditions that can be associated with pulmonary hypertension. When the pressure in the pulmonary blood vessels is too high, blood flows less easily and the heart has to work harder.

In patients with systemic scleroderma, pulmonary hypertension is a concern because its symptoms can develop slowly. Shortness of breath during activity, tiring quickly, dizziness, a heavy feeling in the chest, or leg swelling can appear gradually.

The 2022 ESC/ERS Guidelines state that a multimodal approach is needed when screening for pulmonary arterial hypertension in patients with SSc, combining clinical features, echocardiography, pulmonary function tests, and NT-proBNP. The guidelines also state that annual screening for PAH in patients with SSc is considered sufficient based on current evidence.

06 / Autoimmune Disease

Diagnosing Scleroderma

Scleroderma is not diagnosed from a single complaint, a single photo of the skin, or a single lab result. The doctor needs to combine your symptom history, a physical examination, blood tests, and organ tests as needed.

Symptom History and Physical Examination

The doctor will ask when the symptoms began, which areas of skin have changed, whether your fingers often change color in the cold, and whether there are sores on your fingertips.

In addition, the doctor will also assess other complaints such as shortness of breath, a dry cough, reflux, difficulty swallowing, palpitations, or tiring easily. The physical examination helps assess the pattern of skin hardening, finger swelling, abnormalities in the small blood vessels, signs of joint problems, and signs of involvement of other organs.

There is no single test to diagnose scleroderma. Doctors use a combination of medical history, symptoms, physical examination, and additional tests.

To make your consultation more efficient, you can bring:

• notes on when your fingers change color;
• photos of skin or finger changes if the symptoms come and go;
• a list of the medications you are taking;
• previous blood test results;
• notes on symptoms of shortness of breath, palpitations, or tiring easily;
• your personal or family history of autoimmune disease.

Blood and Antibody Tests

Blood tests can help the doctor assess whether an autoimmune process is present. Some terms that often come up in the evaluation of scleroderma are ANA, anti-centromere, and anti-Scl-70.

However, antibody results must be read together with your symptoms and the clinical examination. NIAMS explains that certain antibodies can be found in some people with scleroderma, but blood tests alone are not enough to make a diagnosis.

So if an anti-centromere or ANA result is positive, this does not automatically mean a person definitely has scleroderma. Instead, the doctor will assess whether the result matches the symptoms, the physical examination, and the findings in other organs.

Lung and Internal Organ Function Tests

Because systemic scleroderma can involve internal organs, additional tests may be needed as indicated.

For the lungs, the doctor may consider:

• pulmonary function tests;
• a DLCO test;
• a chest X-ray;
• an HRCT scan if needed.

The Indonesian Rheumatology Association guideline states that pulmonary function tests such as FVC or DLCO can be used to screen for and monitor interstitial lung disease in systemic sclerosis. HRCT can also be used to assess interstitial lung disease from the time of diagnosis, as indicated.

For other organs, tests can be tailored to the symptoms. For example, a digestive tract evaluation if there is reflux or difficulty swallowing, as well as monitoring of blood pressure and kidney function when needed.

Heart Tests That May Be Recommended

If there are heart and lung complaints or a need for monitoring in systemic scleroderma, the doctor may consider some of the following tests:

In the evaluation of pulmonary hypertension, echocardiography and cardiac catheterization are commonly used tests to measure or estimate the pressure in the pulmonary arteries. ECG and cardiac MRI can also help assess the effects on the heart.

Meanwhile, the Indonesian Rheumatology Association recommendations state that evaluating heart involvement in SSc can include multidisciplinary clinical and supporting examinations, including echocardiography, CMR, ECG, and other tests depending on signs and symptoms.

Learn More:

• ECG (Electrocardiography)
• Echocardiography
• Holter Monitoring
• Right Heart Catheterization
• Cardiovascular Diagnostic Center

07 / Autoimmune Disease

Treatment and Monitoring

Scleroderma treatment aims to control symptoms, slow progression, manage organ involvement, and maintain quality of life. To date, scleroderma is generally managed as a long-term condition.

Can Scleroderma Be Cured?

There is currently no therapy that cures scleroderma completely. However, treatment can help relieve symptoms, limit damage, and slow the progression of the disease.

This statement should be understood calmly. “Not yet completely curable” does not mean nothing can be done. On the contrary, appropriate monitoring and treatment can help patients understand their condition, manage their symptoms, and detect organ involvement earlier.

Therapy to Control Symptoms

Scleroderma therapy depends on the type of disease, the symptoms, and the organs affected. The approach may include:

• therapy for skin complaints;
• therapy for Raynaud’s phenomenon and blood vessel problems;
• therapy for reflux or digestive problems;
• therapy for interstitial lung disease if present;
• therapy for pulmonary hypertension if confirmed;
• therapy for heart rhythm or heart function problems if found.

The American College of Rheumatology explains that scleroderma management can include therapy for Raynaud’s, heartburn, skin involvement, interstitial lung disease, and pulmonary arterial hypertension, tailored to the patient’s clinical manifestations.

Patients are not advised to stop, switch, or add medications on their own. Some scleroderma medications act on the immune system or the blood vessels and require monitoring by a doctor.

The Importance of Long-Term Monitoring

In systemic scleroderma, regular monitoring helps the doctor assess whether there are changes in the skin, lungs, heart, kidneys, digestive tract, or blood vessels.

The Indonesian Rheumatology Association emphasizes that every patient with SSc needs an assessment of organ involvement to determine the appropriate treatment plan.

For active patients, monitoring also helps them recognize their own body’s patterns. For example, whether shortness of breath appears sooner when climbing stairs, whether palpitations happen more often, or whether the fingers hurt more often in the cold.

As a result, planned check-ups are usually more reassuring than waiting for symptoms to become severe.

08 / Autoimmune Disease

When Should You See a Doctor?

It is advisable to consult a doctor if symptoms persist, get worse, or appear together with signs that point to organ involvement. Earlier examination helps distinguish scleroderma from other conditions and determine the right monitoring steps.

Early Signs That Should Be Checked

A person should consult a doctor if they experience:

• skin tightening or thickening without a clear cause;
• fingers that are often cold, numb, or change color;
• sores on the fingertips that are slow to heal;
• swollen or stiff fingers;
• difficulty swallowing;
• severe or persistent reflux;
• unusual fatigue;
• shortness of breath during activity;
• a persistent dry cough;
• recurring palpitations.

The Indonesian Rheumatology Association guideline states that systemic sclerosis should be suspected in patients with skin hardening, pulmonary fibrosis, vascular abnormalities such as ulcers or gangrene, or pulmonary hypertension without another clear cause.

Getting checked earlier does not mean every symptom is dangerous. Rather, an examination helps the doctor understand the cause of the complaints more accurately.

Signs That Call for a Heart and Lung Evaluation

A heart and lung evaluation should be considered if a patient with suspected or diagnosed scleroderma experiences:

• shortness of breath that persists or gets worse;
• tiring more quickly than usual;
• a reduced ability to exercise or be active;
• chest discomfort;
• recurring palpitations;
• dizziness or nearly fainting;
• swelling in the feet;
• a dry cough that does not improve.

In evaluating pulmonary hypertension, the doctor may assess your symptom history, risk factors, physical examination, oxygen saturation, heart and lung sounds, and any swelling in the abdomen or legs.

Seek medical help immediately if you experience severe chest pain, severe shortness of breath, fainting, sudden weakness, or acute symptoms that feel worrying.

Which Doctors Treat Scleroderma?

The main evaluation of scleroderma usually involves an internal medicine doctor or a rheumatology subspecialist. If the skin is the main manifestation, a dermatologist can also play a role.

Because systemic scleroderma can involve many organs, cardiologists, pulmonologists, nephrologists, gastroenterologists, physical medicine and rehabilitation specialists, or other specialists may be involved according to the patient’s needs. Rheumatologists are often involved in scleroderma care, and other specialists can be brought in depending on the organs affected.

At Heartology Cardiovascular Hospital, cardiologists play a role mainly when patients have complaints that point to involvement of the heart, the blood vessels, or the pressure in the pulmonary blood vessels.

09 / Autoimmune Disease

Living With Scleroderma

Living with scleroderma requires understanding, monitoring, and good communication with your medical team. Daily steps do not replace treatment, but they can help patients recognize changes in their condition sooner.

Protect Your Hands From Raynaud’s Triggers

If you have Raynaud’s phenomenon, a few simple steps can help reduce triggers:

• keeping your body and hands warm;
• wearing gloves in cold environments;
• avoiding sudden exposure to cold;
• managing stress;
• protecting your fingers from small injuries;
• quitting smoking if you smoke, because nicotine can narrow the blood vessels.

Patients with scleroderma are advised to keep their bodies warm, avoid cold or wet environments that trigger Raynaud’s, and quit smoking because nicotine can worsen the narrowing of blood vessels.

Monitor Breathing, Chest, and Activity Complaints

For active patients, recording symptoms can help the doctor understand changes in their condition. Simple notes can include:

• when shortness of breath appears;
• whether shortness of breath occurs when climbing stairs or at rest;
• whether palpitations occur during activity, after coffee, during stress, or at rest;
• whether your feet are starting to swell;
• whether a dry cough persists;
• whether daily activities feel harder than usual.

These notes are not for diagnosing yourself. However, the information can make your consultation more focused and efficient.

Keep Communicating With Your Medical Team

Systemic scleroderma can differ from person to person. That is why patients should understand the monitoring plan prepared by their doctor, including when skin, lung, heart, kidney, or digestive tract examinations are needed.

Bring your previous test results to follow-up visits. In addition, ask which signs need to be monitored at home and when to contact your doctor. An approach like this helps patients feel more prepared, not more anxious.

10 / Autoimmune Disease

Conclusion

Scleroderma is an autoimmune disease that can cause the skin to tighten or thicken. In some patients, especially those with systemic scleroderma, the condition can also involve the blood vessels, lungs, heart, kidneys, and digestive tract.

Symptoms such as fingers that often change color, stiff finger skin, difficulty swallowing, shortness of breath, tiring easily, palpitations, chest pain, or leg swelling should not be self-diagnosed right away. At the same time, these complaints should not be ignored if they persist or get worse.

With the right understanding, patients can take calmer steps. A doctor’s examination helps distinguish scleroderma from other conditions, assess possible organ involvement, and determine a monitoring plan that is personal and comprehensive.