What is the definition of Recurrent Respiratory Papillomatosis?
Recurrent respiratory papillomatosis (RRP) is a rare viral disease where tumors (papillomas) grow in the air passages leading from the nose and mouth into the lungs (respiratory tract). There are two types, a juvenile-onset form and an adult-onset form. The tumors can cause a hoarse voice, chronic cough, and difficulty breathing. They may vary in size and grow very quickly, and may grow back even when removed. These tumors rarely become cancerous, but can cause long-term airway and voice complications. RRP is caused by two types of human papillomavirus (HPV), called HPV 6 and HPV 11. It is transmitted from an infected mother to her baby through the placenta or through the birth canal. HPV can also be transmitted from one adult to another through oral sex. RRP is treated by surgical removal of the tumors and sometimes using additional medications. The HPV vaccine can help prevent infection.
What are the alternative names for Recurrent Respiratory Papillomatosis?
- Juvenile laryngeal papilloma
- Laryngeal papilloma, recurrent
- Juvenile-onset recurrent respiratory papillomatosis (type)
- JORRP (type)
- Adult-onset recurrent respiratory papillomatosis (type)
- AORRP (type)
- Respiratory papillomatosis, recurrent
What are the causes for Recurrent Respiratory Papillomatosis?
Most cases of recurrent respiratory papillomatosis (RRP) are caused by two types of human papillomavirus (HPV), HPV 6 and HPV 11.
What are the symptoms for Recurrent Respiratory Papillomatosis?
The following list includes the most common signs and symptoms in people with recurrent respiratory papillomatosis (RRP). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list also does not include every symptom or feature that has been described in this condition.
Symptoms may include:
- Growth of benign (non-cancerous) tumors in the airway
- Voice hoarseness (dysphonia)
- Shortness of breath (dyspnea)
- Chronic cough
Recurrent respiratory papillomatosis (RRP) is a chronic disease, meaning it is a long-term or persistent condition. It can occur in childhood, before age 12, or in adulthood, usually between ages 20-40. Earlier age at onset is associated with more severe symptoms. RRP is very unpredictable. In some people, RRP goes away on its own, while in others, it causes severe, recurring disease. Very rarely, the tumors in RRP can become cancerous and spread.
What are the current treatments for Recurrent Respiratory Papillomatosis?
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. 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.
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.
Specialists who may be involved in the care of someone with RRP include:
- Otolaryngologist (ENT doctor)
- Pulmonologist (lung specialist)
- Infectious disease specialist
How is Recurrent Respiratory Papillomatosis diagnosed?
Recurrent respiratory papillomatosis (RRP) is diagnosed based on a clinical examination, the symptoms, and imaging studies, such as a CT scan. It is confirmed by doing a biopsy, which is removing a small piece of tumor tissue and testing it for features associated with papillomas.
Is Recurrent Respiratory Papillomatosis an inherited disorder?
Recurrent respiratory papillomatosis (RRP) is a viral disease and is not inherited in families. The human papillomavirus (HPV) that causes RRP is passed from an infected mother to a baby through the placenta during pregnancy, or during childbirth through the vaginal canal. It can also be passed from one adult to another through oral sex.
Not everyone who gets HPV will get RRP. Many people have HPV and do not have any symptoms. There is some evidence that genetic factors influence which people with HPV will get sick and how severe the symptoms will be.