Recurrent respiratory papillomatosis

Summary

Recurrent respiratory papillomatosis (MONDO:0018955) is a disease and 26 clinical trials. Top therapeutic interventions include cidofovir anhydrous, propranolol, and avelumab. A subtype of respiratory system disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
• Phenotypes (HPO): 23
• Clinical trials: 26

Clinical features

Epidemiology

Prevalence records

6 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	1-9 / 100 000		Europe	Validated
Annual incidence	1-9 / 1 000 000	0.16	Netherlands	Validated
Annual incidence	1-9 / 100 000	2.34	United States	Validated
Annual incidence	1-9 / 1 000 000	0.76	Denmark	Validated
Point prevalence	1-9 / 100 000	4	Netherlands	Validated
Point prevalence	1-9 / 100 000	1.42	United Kingdom	Validated

Signs & symptoms

Clinical features (HPO)

23 HPO clinical features (Orphanet curated; top 23 by frequency):

HPO ID	Term	Frequency
HP:0001609	Hoarse voice	Very frequent (80-99%)
HP:0001618	Dysphonia	Frequent (30-79%)
HP:0002098	Respiratory distress	Frequent (30-79%)
HP:0002778	Abnormal trachea morphology	Frequent (30-79%)
HP:0001508	Failure to thrive	Occasional (5-29%)
HP:0002015	Dysphagia	Occasional (5-29%)
HP:0002093	Respiratory insufficiency	Occasional (5-29%)
HP:0002094	Dyspnea	Occasional (5-29%)
HP:0002105	Hemoptysis	Occasional (5-29%)
HP:0002781	Upper airway obstruction	Occasional (5-29%)
HP:0002788	Recurrent upper respiratory tract infections	Occasional (5-29%)
HP:0002789	Tachypnea	Occasional (5-29%)
HP:0006532	Recurrent pneumonia	Occasional (5-29%)
HP:0010307	Stridor	Occasional (5-29%)
HP:0030828	Wheezing	Occasional (5-29%)
HP:0031246	Nonproductive cough	Occasional (5-29%)
HP:0001279	Syncope	Very rare (<1-4%)
HP:0001945	Fever	Very rare (<1-4%)
HP:0002088	Abnormal lung morphology	Very rare (<1-4%)
HP:0002779	Tracheomalacia	Very rare (<1-4%)
HP:0002860	Squamous cell carcinoma	Very rare (<1-4%)
HP:0030842	Choking episodes	Very rare (<1-4%)
HP:0100750	Atelectasis	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	recurrent respiratory papillomatosis
Mondo ID	MONDO:0018955
MeSH	C535297
Orphanet	60032
ICD-11	151039887
NCIT	C128637
SNOMED CT	472827002
UMLS	C1168198
MedGen	216998
GARD	0000111
MedDRA	10059314
NORD	1651
Is cancer (heuristic)	no

Also known as: adult-onset recurrent respiratory papillomatosis (type) · AORRP (type) · JORRP (type) · juvenile laryngeal papilloma · juvenile-onset recurrent respiratory papillomatosis (type) · laryngeal papilloma, recurrent · recurrent respiratory papillomatosis · respiratory papillomatosis, recurrent · RRP

Disease family

This is a subtype of respiratory system disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › respiratory system disorder › recurrent respiratory papillomatosis

Related subtypes (58): lower respiratory tract disorder, respiratory system cancer, respiratory system benign neoplasm, allergic respiratory disease, paranasal sinus disorder, upper respiratory tract disorder, pertussis, severe acute respiratory syndrome, sleep apnea syndrome, diaphragm disorder, pulmonary tuberculosis, altitude sickness, perinatal asphyxia, pulmonary nodular lymphoid hyperplasia, tracheobronchopathia osteochondroplastica, Williams-Campbell syndrome, cystic fibrosis, growth delay-hydrocephaly-lung hypoplasia syndrome, laryngo-onycho-cutaneous syndrome, congenital pulmonary lymphangiectasia, familial primary pulmonary hypoplasia, Mounier-Kuhn syndrome, Young syndrome, lung agenesis-heart defect-thumb anomalies syndrome, sudden infant death-dysgenesis of the testes syndrome, alpha 1-antitrypsin deficiency, hereditary sclerosing poikiloderma with tendon and pulmonary involvement, autoimmune interstitial lung disease-arthritis syndrome, mucopolysaccharidosis-plus syndrome, congenital bronchobiliary fistula, bronchogenic cyst, primary ciliary dyskinesia, congenital pulmonary airway malformation, transient hyperammonemia of the newborn, congenital pulmonary sequestration, Siegler-Brewer-Carey syndrome, tracheal agenesis, 16q24.1 microdeletion syndrome, staphylococcal necrotizing pneumonia, pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis, plastic bronchitis, IgG4-related mediastinitis, bronchopulmonary dysplasia, infantile apnea, diffuse alveolar hemorrhage, respiratory or thoracic malformation, pulmonary agenesis, eosinophilic granuloma, disorder of pharynx, respiratory tract neoplasm, pulmonary alveolar proteinosis with hypogammaglobulinemia, respiratory tract infectious disorder, Middle East respiratory syndrome, reactive airway disease, acinar dysplasia, pulmonary hypoplasia, isolated left bronchial isomerism, bronchiectasis and nasal polyposis

Subtypes (1): laryngeal papillomatosis

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 26.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	7
PHASE2	6
PHASE4	3
PHASE1/PHASE2	3
PHASE1	3
EARLY_PHASE1	2
PHASE2/PHASE3	1
PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06538480	PHASE4	RECRUITING	Zopa Retreatment and Vector Shedding in Adults With RRP
NCT00205374	PHASE4	COMPLETED	Use of Cidofovir for Recurrent Respiratory Papillomatosis
NCT00747461	PHASE4	TERMINATED	Interventional Cryotherapy for the Eradication of Benign Airway Disease (ICE the BAD)
NCT01058317	PHASE2/PHASE3	WITHDRAWN	Propranolol Administration in Pediatric Patients With Recurrent Respiratory Papillomatosis
NCT01995721	PHASE3	UNKNOWN	4-valent HPV Vaccine to Treat Recurrent Respiratory Papillomatosis in Children
NCT04724980	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Adjuvant PRGN-2012 in Adult Patients With Recurrent Respiratory Papillomatosis
NCT07339163	PHASE1/PHASE2	NOT_YET_RECRUITING	Efficacy of Booster Injection With Gardasil-9 Vaccine on Intersurgical Interval* for Adults With Recurrent Respiratory Papillomatosis*
NCT00038714	PHASE2	COMPLETED	A Trial Study of SGN-00101 in Treating Pediatric Patients With Recurrent Respiratory Papillomatosis
NCT00571701	PHASE2	COMPLETED	Study of Celebrex (Celecoxib) in Patients With Recurrent Respiratory Papillomatosis
NCT02555800	PHASE2	UNKNOWN	Bevacizumab Versus Cidofovir for the Treatment of Recurrent Respiratory Papillomatosis
NCT02632344	PHASE2	COMPLETED	Pembrolizumab for HPV-associated Recurrent Respiratory Papilloma Patients
NCT02859454	PHASE2	COMPLETED	Avelumab for People With Recurrent Respiratory Papillomatosis
NCT03707587	PHASE2	COMPLETED	M7824 in People With Recurrent Respiratory Papillomatosis
NCT05215249	PHASE1/PHASE2	WITHDRAWN	Optiflow vs. Convential Anesthesia During Pediatric Laryngeal Papilloma Removal
NCT07317154	PHASE1	RECRUITING	Autologous Dendritic Cell Vaccine For Recurrent Respiratory Papillomatosis (RRP) Patients
NCT01020747	PHASE1	COMPLETED	Bevacizumab for Treatment of Recurrent Respiratory Papillomatosis (RRP)
NCT02854761	PHASE1	UNKNOWN	Safety Study of EF-022 in Adults With Recurrent Respiratory Papillomatosis (RRP)
NCT04645602	EARLY_PHASE1	RECRUITING	Merck IIT: RRP Pembro and Lenvatinib
NCT00829608	EARLY_PHASE1	WITHDRAWN	Therapeutic Treatment With Human Papillomavirus Quadrivalent Vaccine for Recurrent Respiratory Papillomatosis
NCT01793168	Not specified	RECRUITING	Rare Disease Patient Registry & Natural History Study - Coordination of Rare Diseases at Sanford
NCT06412172	Not specified	RECRUITING	The Natural History and Biological Study of Pulmonary Recurrent Respiratory Papillomatosis (pRRP)
NCT00550914	Not specified	COMPLETED	Multi-Center, Randomized Trial of the Pulsed Dye Laser for JORRP
NCT02217358	Not specified	COMPLETED	Value of Narrow Band Imaging (NBI) Endoscopy in the Early Diagnosis of Laryngeal Cancer and Precancerous Lesions
NCT02592902	Not specified	COMPLETED	Recurrent Respiratory Papillomatosis and Extraesophageal Reflux
NCT03465280	Not specified	UNKNOWN	Airway Intervention Registry (AIR): Recurrent Respiratory Papillomatosis (RRP)
NCT04513392	Not specified	UNKNOWN	Comparison of BL and KTP Laser for Treatment of Benign Vocal Fold Lesions

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
CIDOFOVIR ANHYDROUS	4	4
PROPRANOLOL	4	3
AVELUMAB	4	1
BINTRAFUSP ALFA	3	1
DEXPROPRANOLOL	2	1