Coughing is a rare but bothersome symptom of pulmonary arterial hypertension (PAH). This long-term disease gradually harms the blood vessels in the lungs. If you or someone you know coughs because of PAH, it’s helpful to know why this happens and ways to address it.

There are many ways to try to relieve your cough, from home remedies to medical treatments, depending on its cause. Here are some helpful tips for managing coughing from PAH. Remember to report any new or worsening symptoms to your pulmonologist (doctor specializing in lung and breathing problems), including PAH cough.

Understanding Coughing With PAH

Before we address tips to treat a cough with PAH, it’s helpful to understand how common it is and why it happens. Coughing might not be the first thing people think of with PAH, but it can affect someone’s daily life. Understanding what causes the cough in PAH can help us find better ways to manage it.

How Common Is Coughing in PAH?

According to the American Lung Association, some of the most common symptoms of PAH include shortness of breath, fatigue, hypertension (high blood pressure), and edema (swelling). Cough and hoarseness are also associated with PAH, though they appear further down the list of common symptoms. As the disease progresses, symptoms become worse. These symptoms are similar to those of other lung diseases, like chronic obstructive lung disease (COPD). They also resemble sarcoidosis, where tiny clumps of inflammatory cells build up, often in the lungs.

Type of Cough in PAH

A PAH cough may be dry or even produce blood. It’s less likely for a PAH cough to produce mucus unless you have another pulmonary disease, such as COPD, or an infection at the same time. “I have emphysema, and I have wheezing, cough, and phlegm,” one myPHteam member said.

This cough may be worse at night than in the daytime, due to your body position. “Is it normal to have persistent night crackles and cough?” one member asked.

Causes of PAH Cough

Although coughing is not a common symptom of PAH, there are several reasons why someone with PAH may experience a cough.

Cough Caused by PAH

A cough is rarely caused by PAH itself. In PAH, the blood vessels in the lungs narrow. This narrowing increases pressure on the heart and in the lungs. In rare cases, the major artery in the lungs — the pulmonary artery — can press directly on the nerve in the chest and cause coughing.

Cough Caused by Medications

Another more common reason for cough in people with PAH is a side effect of medications. One myPHteam member said, “Since starting a new PAH med, I developed a spastic barking cough. I temporarily stopped it, and the cough has almost completely gone away.” For example, bosentan (Tracleer) is an endothelin receptor antagonist for PAH that can cause a cough, sometimes with blood.

Additionally, ACE inhibitors, a class of drugs used to treat heart disease and hypertension, are known to cause cough. Let your doctor know what medications you’re taking, especially any drugs that you started right before your cough began.

Cough Caused by Other Medical Conditions

Another common reason for cough in people with PAH is another underlying disease triggering the cough. Some lung diseases that cause chronic cough (a cough that lasts for more than eight weeks) include:

• COPD
• Asthma
• Cystic fibrosis

Chronic cough can also be caused by conditions that aren’t related to the lungs, such as gastroesophageal reflux disease (GERD, commonly referred to as heartburn), allergies, and sinus problems.

Tips for Managing Coughing With PAH

No matter what’s causing it, coughing is an annoying and sometimes painful symptom to deal with while living with PAH. Here are some practical tips to help you reduce and manage your cough.

1. Discover the Cause of Your Cough

Because a cough can be caused by several different medications and illnesses, including medications to treat PAH and occasionally PAH itself, the first step is to find what’s causing it. See your pulmonologist or primary care doctor if your cough has lasted for many weeks or has disrupted your sleep, ability to eat, hydration, or work. A health care provider can run tests and assess your medical history to figure out what may be causing your cough.

2. Use Home Remedies

Coughing irritates the whole airway, from the lungs to the throat. Here are some home remedies that may help reduce your cough without medication:

• Drinking plenty of water throughout the day can help keep your throat moist.
• Drinking other liquids, such as warm (not too hot) tea with honey, can help coat your throat and soothe irritation. “I learned lemon and honey will calm my cough,” one myPHteam member shared.
• Using a humidifier can help add moisture to the air you breathe at home.

3. Avoid Irritants

Many things may cause a cough — pollen allergies, cigarette smoke, strong smells, and polluted air — are a few potential triggers. One member said their cough became unbearable when they had a viral infection: “I recently got norovirus, and that gave me cough-related syncope [fainting] episodes, even on oxygen 24/7. They are getting worse, and I am concerned.” Staying away from these triggers can help prevent coughing with PAH.

4. Try Breathing Exercises

Try practicing deep breathing exercises to help clear your airways and reduce coughing. Try breathing in slowly through your nose and out through your mouth. This technique can help reduce your cough reflex and open your airways.

Sometimes, positioning your body and head can help reduce the need to cough. “One thing my medical team tells me is to sit down on the floor as soon as you feel a tiny cough coming on, just in case it becomes a bigger coughing fit,” noted a member.

5. Treat Your PAH

If your cough is caused by worsening lung damage from PAH, treating the underlying cause can help reduce cough and other symptoms of pulmonary hypertension. Work with your pulmonologist to find the right PAH medications for your symptoms. Some medicines help with coughing more directly than others, while other medications may be more likely to trigger a cough or make it worse.

Talk to your doctor about what medications are right for you. There are many treatment options for PAH. Health care providers will often prescribe multiple medications to treat PAH, also known as combination therapy.

Classes of drugs that treat PAH include:

• Prostaglandins such as epoprostenol (Flolan)
• Endothelin receptor antagonists such as macitentan (Opsumit)
• Phosphodiesterase-5 inhibitors such as sildenafil
• Soluble guanylate cyclase stimulators such as riociguat (Adempas)

6. Keep a Symptom and Medication Diary

Sometimes, a cough can be related to some of your PAH medications. If you feel like your cough began after you started a new medication, talk to your doctor. If you have a log of when your cough began and when you started a medication, your doctor can help determine whether your treatment may be the source of your cough. If a drug is causing your cough, you may be able to switch doses or to a different treatment.

If you have a cough from another illness alongside PAH, do not take over-the-counter cough medicine or decongestant without consulting your doctor. According to the Pulmonary Hypertension Association, decongestants containing pseudoephedrine and phenylephrine can interact with PAH medications and make PAH symptoms worse.

7. Practice a Healthy Lifestyle

Taking control of your overall health — with a healthy diet, physical activity to the best of your ability, and adequate sleep — can help reduce inflammation and slow PAH disease progression. In turn, you may find yourself experiencing fewer symptoms, such as cough.

8. Know When To Get Emergency Medical Care

Sometimes, coughing with PAH can be a sign of a bigger, potentially life-threatening problem such as blood clots in the lung or poor lung function.

Seek medical help if you:

• Have a severe or worsening cough that makes it hard to breathe or sleep
• Are coughing up blood or pink-tinged mucus
• Feel chest pain or tightness along with coughing
• Suddenly have trouble breathing or start wheezing

Coughing can be a challenging symptom for people with PAH, but there are ways to manage it. By understanding its causes and following these tips, along with getting high-quality health care, you can better cope with coughing and improve your quality of life.

Connect With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 54,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Have you experienced coughing with PH? How have you managed this symptom? Share your experience and tips in the comments below or by posting on myPHteam.