Coughing doesn’t always occur with pulmonary arterial hypertension (PAH), but when it does it can be bothersome. This long-term disease gradually harms lung blood vessels and restricts blood flow due to high blood pressure in the arteries of the lungs. If you or someone you know coughs because of PAH, it’s helpful to know why this happens and how to address it.

You can take steps to relieve a PAH cough — including home remedies and prescription medical treatments — depending on its cause. Here are some helpful tips for managing coughing with PAH. Remember to report any new or worsening symptoms to your pulmonologist (a doctor specializing in lung and breathing problems), including a cough.

Understanding Coughing With PAH

Before thinking about how to treat a cough with PAH, it’s helpful to learn what type of cough may occur with PAH and why it happens. Understanding the cause can help you find better ways to manage it.

How Common Is Coughing With PAH?

Although data on how common a cough is with PAH isn’t readily available, coughing isn’t always listed as a symptom of PAH. According to Cleveland Clinic, the most common symptoms of PAH are blue lips or fingers, chest pressure or pain, dizziness, shortness of breath, racing heartbeat, and edema (swelling).

The American Lung Association points to similar signs of PAH but includes cough and hoarseness on its list of common symptoms. Early symptoms of PAH may be mild. As the disease progresses, symptoms become worse, and some people may develop a cough. Symptoms of PAH are similar to those of other lung diseases, and they can be confused with conditions such as chronic obstructive pulmonary disease (COPD), congestive heart failure, pulmonary fibrosis, and asthma — all of which can include a cough. PAH also resembles sarcoidosis, where tiny clumps of inflammatory cells build up, often in the lungs.

A PAH cough is often dry and may 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 myPHteam member asked.

Causes of Cough in PAH

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 small blood vessels in the lungs narrow. This narrowing increases pressure on the heart and in the larger lung blood vessels. These may consequently enlarge. In rare cases, the major artery in the lungs — the pulmonary artery — can become extremely large, press directly on nerves in the chest, and cause coughing.

Cough Caused by Medications

A 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 is an endothelin receptor antagonist for PAH that can cause pulmonary edema (fluid buildup in the lung) as a side effect with a cough, sometimes with blood.

“Since starting a new PAH med, I developed a spastic barking cough.”

— A myPHteam member

Additionally, ACE inhibitors, a class of drugs used to treat heart disease and hypertension, are known to cause dry 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

People with PAH sometimes cough because another underlying disease is 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.

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.”

Tips for Managing Coughing With PAH

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

1. Discover the Cause of Your Cough

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 ability to sleep, eat, stay hydrated, or work. A healthcare 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.

“I learned lemon and honey will calm my cough.”

— A myPHteam member

3. Avoid Irritants

Many substances can irritate the lungs and airways and may cause a cough. Pollen, cigarette smoke, strong smells or fumes, and polluted air are a few potential triggers. 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, changing the position of 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.

Deep breathing techniques can help reduce your cough reflex and open your airways.

5. Treat Your PAH

If your cough is caused by worsening lung damage from PAH, treating the underlying cause can help reduce coughing 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 which medications are right for you. There are many treatment options for PAH. Healthcare providers will often prescribe multiple medications to treat PAH, also known as combination therapy.

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 adjust your dose or switch to a different treatment.

If you have a cough from another illness alongside PAH, do not take an 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.

Talk to your doctor about a referral for a registered dietitian or physical therapist if you need help planning meals or exercise that is appropriate for you.

8. Know When To Seek Emergency Medical Care

Sometimes, coughing with PAH can be a sign of a potentially life-threatening problem. Coughing could indicate another condition that causes poor lung function, a pulmonary embolism (blood clot in the lung), or lung cancer.

Seek medical help if you:

• Have a severe or worsening cough that makes it hard to breathe or sleep
• Cough 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 healthcare, 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 56,000 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.