eMedicine Specialties > Pulmonology > Lung Tumors

Recurrent Respiratory Papillomatosis

Author: Eloise M Harman, MD, Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, University of Florida College of Medicine
Contributor Information and Disclosures

Updated: Mar 6, 2008

Introduction

Background

Recurrent respiratory papillomatosis (RRP) is a disease caused by the human papillomavirus (HPV). See Human Papillomavirus and Medscape’s HPV and Cervical Cancer Resource Center for more information on HPV. Warty growths in the upper airway may cause significant airway obstruction or voice change. RRP has a bimodal age distribution and manifests most commonly in children younger than 5 years (juvenile-onset RRP [JORRP]) or in persons in the fourth decade of life (adult-onset RRP [AORRP]).

JORRP is more common and more severe than AORRP. JORRP is caused by exposure to HPV during the peripartum period. Researchers do not know with certainty the manner in which adult patients acquire the virus that causes AORRP, but sexual transmission is likely.

Treatment usually involves repeated debulking of the warty growths by angiolytic laser or microdebridement coupled with intralesional cidofovir therapy in patients with moderate or severe disease. Interferon treatment appears to slow the rate of growth without curing the disease. Although some antiviral agents (eg, cidofovir) also may slow the rate of regrowth of lesions, they are not curative. Eventually, some patients may enter remission. In 3-5% of patients, respiratory papillomas may undergo malignant degeneration to squamous cell carcinoma, and the prognosis for patients with these cancers is quite poor. See Squamous Cell Carcinoma for more information on this topic.

A few related Medscape CME courses are as follows:

Pathophysiology

HPV, the virus associated with cutaneous warts, genital condyloma, and cervical cancer, causes RRP. While more than 20 types of HPV can cause genital warts, only 2 of these, HPV-6 and HPV-11, cause the vast majority of cases of RRP. The disease associated with HPV-11 is more severe; thus, in children with HPV-11–associated disease, as many as 70% may require tracheostomy, compared with less than 20% of children infected with HPV-6 (see Human Papillomavirus).

The cause of JORRP is peripartum transmission of the virus from an infected mother. Vaginal delivery is a risk factor, but cesarean delivery is not completely protective.1 The classic triad for increased risk of JORRP includes being firstborn, vaginal delivery, and having a mother younger than 20 years.2 Suspect sexual abuse in children older than 5 years who acquire RRP. The mode of transmission of the virus in adults with RRP is unknown, but sexual transmission is probable.

Papillomas may develop anywhere in the respiratory tract, from the nose to the lung; however, 95% of cases involve the larynx. The sites of respiratory system involvement have been described more completely for JORRP; 52% of children have only laryngeal involvement. The trachea is the next most commonly involved site. However, 31.8% of children had papillomas in areas outside of the trachea and larynx (eg, oropharynx, nasopharynx, mouth, bronchi, lung parenchyma).

Frequency

United States

In 1993, survey results estimated 5977 new cases of RRP occur each year in the United States—2354 new pediatric cases and 3623 new adult cases. The estimated incidence in children aged 14 years or younger is 4.3 cases per 100,000 population. In adolescents older than 15 years, the incidence is 1.8 cases per 100,000 population.3

International

Researchers in Denmark have studied the incidence of JORRP and concluded that the incidence in Denmark is the same as that in the United States.

Mortality/Morbidity

  • Because the disease is uncommon and requires direct laryngoscopy for diagnosis, children usually have symptoms for a year before a physician makes the diagnosis. The morbidity of this disease has been studied more completely for JORRP, in which the average number of surgical procedures required is 4.4 per child per year and the average number of procedures per child's lifetime is more than 20.4 This exacts a tremendous financial cost and severely affects quality of life, including the ability to attend school and work. Ten to 15% of children with JORRP ultimately require tracheostomy, usually when younger than 2 years. Many eventually tolerate decannulation.
  • The need for tracheostomy in adults appears to be less common than in children, but repeated surgical procedures are the rule, and procedures may be required as often as every few weeks.
  • Malignant degeneration of papillomatous lesions to squamous cell carcinoma occurs in 3-5% of patients with RRP.

Race

Initial results from the National Registry for Juvenile-Onset Recurrent Respiratory Papillomatosis,3 a study encompassing 399 children from 20 tertiary care pediatric otolaryngology centers, indicate that 62.7% of the children were white, 28.3% were black, and 2.3% were Asian or Native American.

Sex

JORRP affects males and females in equal numbers, whereas AORRP is more common in males.

Age

The mean age at diagnosis of JORRP is 3.8 years. The adult form usually manifests in the third or fourth decade of life but may rarely manifest in patients older than 60 years.

Clinical

History

RRP is a rare disease, and adult patients may have symptoms for months or longer before the disease is recognized. Because the larynx is the most frequently affected site for both JORRP and AORRP, symptoms of upper airway obstruction predominate. Upper airway obstruction may be life threatening and may be the presenting symptom. Hoarseness is the most common presenting symptom. Other symptoms include the following:

  • Voice change
  • Choking episodes
  • Foreign body sensation in the throat
  • Cough
  • Dyspnea
  • Inspiratory wheeze
  • Stridor

Physical

Physical findings often are nonspecific. Voice change may be noted. Inspiratory wheezing, stridor, or both may develop over the trachea or the upper thorax.

Patients with JORRP commonly present with a weak cry, episodes of choking, hoarseness, or failure to thrive. Patients with AORRP present with hoarseness, choking spells, voice change, dyspnea, or a foreign body sensation in the throat.

Causes

HPV causes RRP. HPV-6 and HPV-11 are the most common types associated with RRP, but, rarely, affected tissues contain HPV-16 and HPV-18. HPV is the most common sexually transmitted disease in the United States; as many as 75% of women have genital HPV at some time in their lives. Thirty to 60% of mothers of children affected with JORRP have genital HPV, compared with 5% of mothers of unaffected children. A study using questionnaires of affected children or their parents (identified through the RRP Foundation) verified that the 3 risk factors for JORRP are (1) firstborn child, (2) vaginal delivery, and (3) mother younger than 20 years. The risk factors for JORRP do not apply to adult-onset cases. This suggests that adult disease does not represent reactivation of latent disease. The mode of transmission of HPV in AORRP is not known. Child-to-parent transmission by cough has never been documented. Sexual transmission is likely.

More on Recurrent Respiratory Papillomatosis

Overview: Recurrent Respiratory Papillomatosis
Differential Diagnoses & Workup: Recurrent Respiratory Papillomatosis
Treatment & Medication: Recurrent Respiratory Papillomatosis
Follow-up: Recurrent Respiratory Papillomatosis
Multimedia: Recurrent Respiratory Papillomatosis
References
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References

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Further Reading

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Keywords

RRP, juvenile-onset recurrent respiratory papillomatosis, JORRP, adult-onset recurrent respiratory papillomatosis, AORRP, human papilloma virus, HPV, human papillomavirus, airway obstruction, voice change, warts, sexually transmitted diseases, STDs

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Contributor Information and Disclosures

Author

Eloise M Harman, MD, Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, University of Florida College of Medicine
Eloise M Harman, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Chest Physicians, American Medical Women's Association, American Thoracic Society, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Harold L Manning, MD, Associate Professor, Departments of Medicine, Anesthesiology and Physiology, Section of Pulmonary and Critical Care Medicine, Dartmouth Medical School
Harold L Manning, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
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