Warning signs of pulmonary hypertension (high blood pressure in the lung arteries) in people with scleroderma can include new or worsening shortness of breath during activity, unusual tiredness, chest discomfort, dizziness or fainting, a racing heartbeat, and swelling in the legs or belly. Symptoms may build slowly, and some people feel normal early on. Regular screening matters even when no warning signs are obvious.
Scleroderma, also called systemic sclerosis when it affects internal organs, can damage blood vessels and the lungs. Pulmonary hypertension is a possible complication that places extra strain on the right side of the heart. Recognizing changes in breathing, energy, and physical ability can help people seek assessment promptly, but symptoms alone cannot establish whether pulmonary hypertension is present.
Which warning signs should people with scleroderma watch for?
The symptoms of pulmonary hypertension may be subtle at first. They can develop gradually and become more noticeable as everyday activities become harder. According to the Pulmonary Hypertension Association, unexplained changes in physical ability are important to report in people with scleroderma, particularly when they affect exercise or normal daily activities.
- Shortness of breath during activity: Walking, climbing stairs, shopping, or doing household chores may leave someone more breathless than before. Breathlessness that is new, worsening, or occurring during activities that were previously manageable deserves medical attention.
- Unusual tiredness or reduced stamina: A person may feel exhausted after routine tasks, need more breaks, or notice that walking distances have become shorter. Fatigue can have many causes, but a persistent decline in energy alongside breathing changes should be discussed with a healthcare professional.
- Chest discomfort or pressure: Some people experience discomfort or pain in the chest, particularly during physical activity. Chest symptoms should not automatically be attributed to scleroderma or assumed to result from muscle or joint problems.
- Dizziness, lightheadedness, or fainting: Reduced tolerance for activity accompanied by lightheadedness or a fainting episode may be concerning. Fainting during exertion warrants urgent medical assessment.
- A racing or pounding heartbeat: A heartbeat that feels unusually fast or forceful may occur with pulmonary hypertension. This symptom can also be associated with other medical conditions, so its cause needs to be assessed in context.
- Swelling in the legs, ankles, or abdomen: Fluid may collect in the lower legs or belly when the right side of the heart struggles to work against increased pressure in the lung circulation. New or increasing swelling should be reported promptly.
- Blue or gray discoloration of the lips or skin: This can occur when oxygen levels are low. New discoloration, especially when accompanied by breathing difficulty, needs urgent assessment.
According to Mayo Clinic, pulmonary hypertension symptoms can include breathlessness, chest pain, dizziness, fatigue, a fast heartbeat, and swelling. These symptoms overlap with many other heart and lung problems, so their presence does not identify the underlying cause.
Can early pulmonary hypertension develop without obvious symptoms?
Yes. Some people with scleroderma have lung or blood vessel involvement before they notice a clear change in how they feel. Others gradually adjust their routines to avoid breathlessness or fatigue, such as taking lifts instead of stairs or resting more often. These changes may be easy to overlook unless they are compared with previous activity levels.
The Johns Hopkins Scleroderma Center explains that lung involvement can be difficult to detect from symptoms or a routine physical examination alone. For this reason, feeling well does not remove the need for recommended monitoring. People with systemic sclerosis should discuss an appropriate screening schedule with their healthcare team, even when they have no noticeable breathing problems.
How is pulmonary hypertension different from other scleroderma-related lung problems?
Scleroderma can affect the lungs in more than one way. Pulmonary arterial hypertension occurs when the arteries carrying blood through the lungs become narrowed or stiff, increasing the pressure the heart must overcome to pump blood. Interstitial lung disease, another possible complication, affects lung tissue and can cause scarring that makes breathing and oxygen exchange more difficult.
Both conditions may cause shortness of breath, fatigue, or reduced exercise tolerance. They can also occur together, and pulmonary hypertension may arise from different underlying causes in people with scleroderma. Symptoms alone cannot reliably distinguish between these conditions. A healthcare professional considers the person's medical history, examination, changes over time, and appropriate test results to investigate the cause.
When should you seek urgent medical attention?
Contact your treating clinician promptly if you develop new or worsening breathlessness, unexplained loss of stamina, recurring dizziness during activity, chest discomfort, a racing heartbeat, or swelling in the legs or abdomen. Tell your rheumatologist or primary care clinician about changes even if you have previously had reassuring screening results. New symptoms may warrant assessment before the next scheduled appointment.
Seek emergency medical care if you experience chest pain with shortness of breath, sudden or severe difficulty breathing, or fainting accompanied by concerning symptoms. Blue or gray lips or skin with breathing difficulty are also an emergency warning sign. The National Heart, Lung, and Blood Institute advises seeking emergency help for chest pain accompanied by shortness of breath because these symptoms can indicate a heart attack or a blood clot in the lungs.
What screening tests can help detect pulmonary hypertension?
Because warning signs may be absent or difficult to distinguish from other scleroderma complications, clinicians use screening and diagnostic tests as well as symptom history. The specific tests depend on the person's circumstances and previous results.
- Echocardiogram: An ultrasound examination of the heart that helps assess heart structure and function and estimate the likelihood of elevated pressure in the lung circulation.
- Pulmonary function tests: Breathing tests that measure aspects of lung function. They can help identify lung abnormalities and changes that require further investigation.
- Blood tests: Tests such as BNP or NT-proBNP may help identify strain on the heart and contribute to the overall assessment.
- Other imaging or heart tests: Depending on the findings, clinicians may recommend an electrocardiogram, chest imaging, or a ventilation-perfusion scan to evaluate other possible causes.
- Right heart catheterization: When pulmonary hypertension is suspected, this procedure measures pressure directly inside the heart and pulmonary arteries. It can confirm the diagnosis and help clarify the type of pulmonary hypertension.
According to Mayo Clinic's diagnostic guidance, an echocardiogram can help identify suspected pulmonary hypertension, while right heart catheterization may be used to confirm it. No single screening test or symptom should be interpreted in isolation; clinicians assess the results together to determine whether additional evaluation is needed.
How often should people with scleroderma be screened?
The Pulmonary Hypertension Association recommends annual screening for pulmonary arterial hypertension in people with scleroderma, including those who do not have symptoms. The appropriate screening plan should be discussed with the clinician managing systemic sclerosis, because the choice and timing of tests depend on the individual's medical history and findings.
Between appointments, it can be useful to note changes in walking distance, stair climbing, energy, dizziness, swelling, or recovery after activity. Share these changes with your healthcare team rather than reducing activity and assuming the change is simply part of living with scleroderma. Do not wait for the next routine screening visit if symptoms are new or getting worse.
Early assessment can help clinicians identify pulmonary hypertension and distinguish it from other conditions that may affect people with scleroderma. Although warning signs are not proof of pulmonary hypertension, noticing changes and keeping up with recommended screening are important parts of ongoing care.