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Recurrent respiratory papillomatosis (RRP)

Recurrent respiratory papillomatosis (RRP) is a rare viral disease where tumors (papillomas) grow in the respiratory tract

Prevalence

1-9 / 100,000

3,310 - 29,790

US Estimated

1-9 / 100,000

5,135 - 46,215

Europe Estimated

Age of Onset

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ICD-10

-

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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5 Facts you should know

FACT

1

The tumors are caused by human papillomavirus (HPV) infection of the throat.

FACT

2

The tumors may lead to narrowing of the airway, which may cause vocal changes or airway obstruction.

FACT

3

There are two types of RRP - juvenile-onset and adult-onset.

FACT

4

They may vary in size and grow very quickly, and may grow back even when removed.

FACT

5

These tumors rarely become cancerous, but can cause long-term airway and voice complications.

Recurrent respiratory papillomatosis (RRP)

Recurrent respiratory papillomatosis

RRP; Juvenile laryngeal papilloma; Laryngeal papilloma, recurrent; Juvenile-onset recurrent respiratory papillomatosis (type); JORRP (type); Adult-onset recurrent respiratory papillomatosis (type); AORRP

What’s your rare IQ?

Recurrent respiratory papillomatosis is caused by which two HPV viruses?

Common Signs & Symptoms

Dysphonia

Voice hoarseness

Benign tumors in the airway

(non-cancerous)

Dyspnea

Shortness of breath

Chronic cough

Respiratory distress

Breathing difficulties
Difficulty breathing

Increased proportion of transitional B cells

Failure to thrive

Faltering weight
Weight faltering

Hemoptysis

Coughing up blood

Current treatments

There is no cure for recurrent respiratory papillomatosis (RRP). Surgery is the primary method for removing tumors to keep the airway open and maintain the voice.[2][3] Because the tumors often grow back, it is common for affected individuals to require repeat surgery. In the most extreme cases where tumor growth is aggressive, a tracheostomy may be performed.[2][3]

About 20% of people with RRP will need adjuvant therapies—therapies that are used in addition to surgery. These include antiviral and anti-tumor medications. The HPV vaccine helps to prevent infection and spread of the virus. There is some evidence that the HPV vaccine may reduce the severity of symptoms in people with RRP.[5]

Specialists who may be involved in the care of someone with RRP include:

    • Otolaryngologist (ENT doctor)
    • Pulmonologist (lung specialist)
    • Surgeon
    • Infectious disease specialist

Top clinical studies

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Top treatments in development

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