Recurrent respiratory papillomatosis

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Other Names:

RRP; Juvenile laryngeal papilloma; Laryngeal papilloma, recurrent; RRP; 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 See More

Categories:

Ear, Nose, and Throat Diseases; Lung Diseases

Summary Summary

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.^[1]^[2]^[3]^[4]

Last updated: 4/28/2020

Symptoms Symptoms

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:^[1]^[3]

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.^[1]^[3]^[4]

Last updated: 4/28/2020

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 20 |

Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Hoarse voice	Hoarseness Husky voice [ more ]	0001609
30%-79% of people have these symptoms
Dysphonia	Inability to produce voice sounds	0001618
Respiratory distress	Breathing difficulties Difficulty breathing [ more ]	0002098
5%-29% of people have these symptoms
Dysphagia	Poor swallowing Swallowing difficulties Swallowing difficulty [ more ]	0002015
Failure to thrive	Faltering weight Weight faltering [ more ]	0001508
Hemoptysis	Coughing up blood	0002105
Nonproductive cough	Dry cough	0031246
Recurrent pneumonia		0006532
Recurrent upper respiratory tract infections	Recurrent colds	0002788
Respiratory insufficiency	Respiratory impairment	0002093
Stridor		0010307
Tachypnea	Increased respiratory rate or depth of breathing	0002789
Upper airway obstruction		0002781
Wheezing		0030828
1%-4% of people have these symptoms
Atelectasis	Partial or complete collapse of part or entire lung	0100750
Choking episodes		0030842
Fever		0001945
Squamous cell carcinoma		0002860
Syncope	Fainting spell	0001279
Tracheomalacia	Floppy windpipe	0002779

Showing of 20 |

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Last updated: 1/1/2021

Cause Cause

Most cases of recurrent respiratory papillomatosis (RRP) are caused by two types of human papillomavirus (HPV), HPV 6 and HPV 11.^[1]^[3]

Last updated: 4/28/2020

Inheritance Inheritance

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.^[1]^[2]^[3]

Last updated: 4/28/2020

Diagnosis Diagnosis

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.^[1]^[2]

Last updated: 4/28/2020

Treatment Treatment

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

Last updated: 4/28/2020

Statistics Statistics

It is estimated that approximately 4 in 100,000 children and 2 in 100,000 adults develop recurrent respiratory papillomatosis in the United States. The incidence of this condition may be decreasing with increased use of the HPV vaccine.^[2]^[6]

Last updated: 4/28/2020

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Related Diseases Related Diseases

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
The differential diagnosis includes acute laryngitis, upper respiratory tract infection, asthma, bronchitis, and other benign or malignant laryngeal tumors. Visit the Orphanet disease page for more information.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Recurrent respiratory papillomatosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

HPV Alliance
PO Box 230575
New York, NY 10023
E-mail: https://hpvalliance.org/pages/contact-us
Website: https://hpvalliance.org/
Recurrent Respiratory Papillomatosis Foundation (RRPF)
P.O. Box 6643
Lawrenceville, NJ 08648
Telephone: 609-530-1095
Fax: 1-866-498-7559
E-mail: bills@rrpf.org
Website: http://www.rrpf.org/

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Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The National Institute on Deafness and Other Communication Disorders (NIDCD) conducts and supports biomedical and behavioral research and research training in the normal and disordered processes of hearing, balance, smell, taste, voice, speech, and language. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Recurrent respiratory papillomatosis. Click on the link to view a sample search on this topic.

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References References

Recurrent Respiratory Papillomatosis. National Organization for Rare Disorders (NORD). Updated 2019; https://rarediseases.org/rare-diseases/recurrent-respiratory-papillomatosis/.
Fortes HR, von Ranke FM, Escuissato DL, Neto CAA, Zanetti G, et al.. Recurrent respiratory papillomatosis: A state-of-the-art review.. Respir Med. May 2017; 126:116-121. https://pubmed.ncbi.nlm.nih.gov/28427542.
Derkay CS, Bluher AE. Update on Recurrent Respiratory Papillomatosis. Otolaryngol Clin North Am. May 2019; 52(4):669-679. https://pubmed.ncbi.nlm.nih.gov/31078306.
Buchinsky FJ, Valentino WL, Ruszkay N, Powell E, Derkay CS, Seedat RY, Uloza V, Dikkers FG et al. Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis.. PLoS One. 2019; 14(6):e0216697. https://pubmed.ncbi.nlm.nih.gov/31194767.
Rosenberg T, Philipsen BB, Mehlum CS, Dyvrig AK, Wehberg S et al. Therapeutic Use of the Human Papillomavirus Vaccine on Recurrent Respiratory Papillomatosis: A Systematic Review and Meta-Analysis. J Infect Dis. Mar 15, 2019; 219(7):1016-1025. https://pubmed.ncbi.nlm.nih.gov/30358875.
Ivancic R, Iqbal H, deSilva B, Pan Q, Matrka L. Current and future management of recurrent respiratory papillomatosis. Laryngoscope Investig Otolaryngol. 2018; 3(1):22-34. https://pubmed.ncbi.nlm.nih.gov/29492465.