Recurrent Respiratory Papillomatosis

Recurrent respiratory papillomatosis (RRP) causes wart-like tumors (papillomas) in your respiratory system. The tumors usually grow on and around your vocal cords in your voice box (larynx). Healthcare providers may call this laryngeal papillomatosis. The tumors can sometimes spread down toward other areas of your respiratory system. These include:

• The long tube that connects your voice box to your bronchi (trachea)
• The large tubes that carry air from your windpipe to your lungs (bronchi)
• Your lungs

Rarely, these tumors can progress to cancer for unclear reasons. They can grow quickly and can come back (recur), even with treatment.

Recurrent respiratory papillomatosis (pronounced “ri-KUR-uhnt” “RES-per-uh-tawr-ee” “pap-uh-LOH-muh-TOH-sis”) can affect anyone. Experts estimate it affects a little over 4 out of 100,000 children and about 2 out of 100,000 adults.

RRP is a serious condition. It can be life-threatening if tumors block your airways. Blocked airways can prevent air from entering your lungs.

Types

Healthcare providers classify recurrent respiratory papillomatosis according to age:

• Juvenile-onset recurrent respiratory papillomatosis (JORRP): Providers usually diagnose JORRP before age 5. It’s usually more aggressive than AORRP. It’s also more likely to come back after treatment.
• Adult-onset recurrent respiratory papillomatosis (AORRP): This usually occurs after age 14. It’s not as common as the RRP that affects children.

Symptoms of recurrent respiratory papillomatosis

It depends on where the respiratory papillomas grow in your respiratory system. As they grow on your vocal cords, they can cause speaking problems. But if they grow very large, they can affect your breathing and swallowing. The most common recurrent respiratory papillomatosis symptoms include:

• Raspy voice (hoarseness)
• Noisy or high-pitched breathing (stridor)
• Rapid breathing (tachypnea)
• Difficulty breathing
• Long-term cough
• Difficulty swallowing (dysphagia)
• Snoring

Other RRP symptoms may include:

• Choking a lot
• Pneumonia that goes away and comes back
• Children growing more slowly than expected

What causes recurrent respiratory papillomatosis?

Human papillomavirus (HPV) causes recurrent respiratory papillomatosis. There are over 100 different strains of HPV. But experts believe HPV 6 and HPV 11 most commonly cause RRP. HPV 11 may also cause more aggressive tumors. These tumors are more likely to affect your breathing and spread to your lungs.

HPV infection is very common. Usually, the infection goes away on its own without major issues. Experts aren’t sure why HPV leads to problems like RRP in some people but not others.

Is it an STD?

Yes and no. HPV causes recurrent respiratory papillomatosis. HPV is the most common STI in the U.S. It spreads easily through anal, oral and vaginal sex. You can spread the HPV that causes RRP through sex. But research doesn’t indicate that RRP itself is contagious.

Experts are still learning a lot about HPV transmission in RRP. In some cases, children can get it from their mother. It can happen during vaginal delivery if the mother has active genital warts. It can also sometimes happen during a C-section. This suggests that a fetus can get HPV before birth. Providers should also consider sexual abuse as a cause in children who experience it.

Experts also suspect that adults with RRP had HPV exposure during their birth. The HPV then “hid” in healthy cells. Later, something activates the hidden HPV, which leads to adult-onset RRP.

Complications

In severe RRP cases, tumors can block your airways. If you can’t breathe, a surgeon may need to use a laser or electrocautery to destroy the tumor. They may also need to make a new way for your lungs to get oxygen (tracheostomy). A tracheostomy affects your ability to speak. Healthcare providers can help you learn how to speak again.

In rare cases, RRP can also progress to laryngeal cancer or lung cancer. Experts aren’t sure why RRP sometimes becomes cancer. But they believe it’s more likely to happen if tumors spread to the lower areas of your respiratory system. Having RRP doesn’t mean you’ll get cancer. But you may need to schedule regular appointments with your provider to keep an eye on it.

How doctors diagnose recurrent respiratory papillomatosis

Your healthcare provider will review your health history and ask about your symptoms. They’ll also look at the back of your throat with a light and a mirror. If they suspect recurrent respiratory papillomatosis, they’ll recommend additional tests. They may also refer you to an ear, nose and throat specialist (otolaryngologist) and a lung specialist (pulmonologist).

Tests that are used

Your healthcare provider may perform the following tests to help diagnose RRP:

• Laryngoscopy: During a laryngoscopy, they’ll numb the inside of your nose and throat. Then, they’ll use a flexible scope to look around your respiratory system for tumors.
• Biopsy: They’ll collect a small tissue sample from any tumors they see. They’ll test the sample for HPV.

How is recurrent respiratory papillomatosis treated?

There’s no cure for recurrent respiratory papillomatosis. But procedures can help remove tumors and keep them from coming back. During the procedures, your healthcare provider will carefully remove the tumors. The options include:

• Laser surgery: Your provider uses beams of light to shrink or destroy tumors.
• Microdebrider: This treatment uses a tool that gently pulls tumors toward a special blade. The blade quickly removes the tumor.
• Cryoablation: Your provider uses extreme cold to destroy tumors.

RRP tumors often return. Some people only need treatment once a year or even less often. But others may need it every few weeks. Children are more likely to have the tumors come back quickly.

If tumors come back fast or cause other symptoms, you may need medications as well. Common medications include:

• Cidofovir: This is an antiviral drug that prevents the HPV virus from replicating.
• Pembrolizumab: This is an immunotherapy agent that uses your immune system to destroy tumor cells.

These medications help prevent tumor growth.

Can the HPV vaccine help treat it?

Yes. The HPV vaccine may slow tumor growth and reduce your need for frequent procedures. If you have any questions about the vaccine, reach out to a healthcare provider.

When should I see my healthcare provider?

RRP shares symptoms with many other common conditions, like asthma and allergies. Talk to a provider if you have RRP symptoms and they don’t go away or if over-the-counter medications don’t help.

During your appointment, you may want to ask:

• What tests do you suggest to diagnose RRP?
• What are the signs that it may be getting worse?
• What treatment do you recommend?
• How often will I need treatment?
• If I have HPV, what are the chances my child will develop RRP?

If you have RRP and are having trouble breathing, call 911 or emergency services.

What can I expect if I have recurrent respiratory papillomatosis?

The outlook for most children and adults is good. Treatment can manage tumors so they don’t interfere with your daily activities. Severe complications aren’t common.

If you or your child has RRP, your provider may recommend monitoring the condition. They may also recommend developing a treatment plan. Follow their instructions and attend all of your appointments. This can help you manage RRP and prevent life-threatening airway blockages.

Can recurrent respiratory papillomatosis be prevented?

The easiest way to prevent recurrent respiratory papillomatosis is to avoid HPV. HPV most commonly spreads through skin-to-skin contact during sexual intercourse. So, the easiest way for many to prevent RRP is to abstain from sex. If you’re sexually active, the HPV vaccine is effective at preventing RRP.

You can also help lower your risk of developing RRP by:

• Getting an STI test before having sex with any new partners
• Limiting the number of your sexual partners
• Practicing safe sex by using dental dams and condoms each time you have sex (but be aware that protection won’t help if genital warts are on areas that condoms don’t cover)

Talk to your provider if you have genital warts and are pregnant or thinking about becoming pregnant. The risk of transmission from you to your baby is low. And there are many safe treatment options if you decide to treat genital warts during pregnancy.