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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
Age of Onset
ICD-10
-
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
The tumors are caused by human papillomavirus (HPV) infection of the throat.
FACT
The tumors may lead to narrowing of the airway, which may cause vocal changes or airway obstruction.
FACT
There are two types of RRP - juvenile-onset and adult-onset.
FACT
They may vary in size and grow very quickly, and may grow back even when removed.
FACT
These tumors rarely become cancerous, but can cause long-term airway and voice complications.
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Common Signs & Symptoms
Dysphonia
Voice hoarseness
Benign tumors in the airway
(non-cancerous)
Dyspnea
Shortness of breath
Chronic cough
Respiratory distress
Increased proportion of transitional B cells
Failure to thrive
Hemoptysis
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