Rare Pulmonology News
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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
Rare View
Laryngeal papillomatosis, also known as recurrent respiratory papillomatosis (RRP) or glottal papillomatosis, is a rare medical condition in which benign tumors (papilloma) form along the aerodigestive tract. The tumors are caused by human papillomavirus (HPV) infection of the throat, most frequently types 6 and 11. The disease occurs in two forms, juvenile and adult papillomatosis, based on whether it develops before or after 20 years of age.†
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
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| INO-3107 With Electroporation (EP) in Participants With HPV-6- and/or HPV-11-Associated Recurrent Respiratory Papillomatosis (RRP) | This is a Phase 1/2 open-label, multicenter trial to evaluate the safety, tolerability, immunogenicity, and efficacy of INO-3107 in subjects with HPV-6 and/or HPV-11-associated recurrent respiratory papillomatosis (RRP). The trial population will include participants who have been diagnosed with either Juvenile-Onset RRP (J-O RRP) as defined by age at first diagnosis <12 years or with Adult- Onset RRP (A-O RRP) as defined by age at first diagnosis ≥12 years. A safety run-in will be performed with up to six participants with a one week waiting period between each enrolled participant. | Phase 1|Phase 2 | Recruiting | Drug: INO-3107|Device: CELLECTRA™ 2000 | Learn more |
| Adjuvant PRGN-2012 in Adult Patients With Recurrent Respiratory Papillomatosis | Recurrent respiratory papillomatosis (RRP) is a rare disease. It is caused by the human papillomavirus (HPV). RRP affects the upper and lower respiratory tracts. It is difficult to treat and can be fatal. Researchers want to see if PRGN-2012 can help. The stated objective of this study is to find a safe, tolerable dose of PRGN-2012 and to see if it works in treating RRP. | Phase 1 | Recruiting | Drug: PRGN-2012 | Learn more |
| M7824 in People With Recurrent Respiratory Papillomatosis | Programmed cell death protein 1 (PD-L1) is a protein on the surface of cells. It regulates if a cell can be killed by immune system cells. It is thought to be able to affect the immune system response to diseased cells like those infected with a virus. The molecule M7824 interferes with the activity of PD-L1. It could help the immune system kill cells infected with a virus . Since recurrent respiratory papillomatosis is caused by a virus infection, this molecule could help. The stated objective of this study is to see if M7824 works in treating recurrent respiratory papillomatosis. | Phase 2 | Active, not recruiting | Drug: M7824 | Learn more |
| Avelumab for People With Recurrent Respiratory Papillomatosis | Recurrent respiratory papillomatosis (RRP) is a rare disease caused by human papillomavirus. It can cause voice changes, airway compromise, pulmonary lesions, and cancers. Programmed death-ligand 1 (PD-L1) is a protein on cells. It may be able to stop or decrease the immune system s response to viruses such as human papillomavirus. The drug Avelumab interferes with the activity of PD-L1. Researchers want to see if Avelumab treats RRP and study its side effects. The stated objective of this study is to see if Avelumab works in treating RRP and is safe. | Phase 2 | Active, not recruiting | Drug: Avelumab | Learn more |
| Pembrolizumab for HPV-associated Recurrent Respiratory Papilloma Patients With Laryngeal, Tracheal and/or Pulmonary Involvement | This research study is evaluating an immune modulatory agent as a possible treatment for patients with Recurrent Respiratory Papillomatosis (RRP) with significant disease involving the larynx, trachea, and/or lungs. The investigators will be using Pembrolizumab as the treating agent. | Phase 2 | Active, not recruiting | Drug: Pembrolizumab | Learn more |
| Lenvatinib and Pembrolizumab Combination Therapy In HPV-associated Recurrent Respiratory Papilloma Patients With Laryngeal, Tracheal, and/or Pulmonary Involvement | This research study is studying Lenvatinib in combination with Pembrolizumab in people with human papillomavirus (HPV)-associated recurrent respiratory papillomatosis (RRP). | Phase 2 | Not yet recruiting | Drug: Lenvatinib|Drug: Pembrolizumab | Learn more |
Top Treatments in Research
| Agent | Class/Mechanism of Action | Development Status | Company | Clinical Studies | More Information |
|---|---|---|---|---|---|
| PRGN-2012 | PRGN-2012 is an innovative therapeutic vaccine with optimized antigen design that uses Precigen’s gorilla adenovector technology, part of Precigen’s proprietary AdenoVerse platform, to elicit immune responses directed against cells infected with human papillomavirus (HPV) type 6 (HPV 6) or HPV type 11 (HPV 11) | Phase 1 | Precigen, Inc. - PRGN-2012 is being developed through a Cooperative Research and Development Agreement (CRADA) with NCI’s Center for Cancer Research (CCR) and Precigen Inc. | More Info | Learn more |
| Pembrolizumab | Pembrolizumab binds to the PD-1 receptor, blocking both immune-suppressing ligands, PD‑L1 and PD‑L2, from interacting with PD-1 to help restore T-cell response and immune response. | Phase 2 | Pembrolizumab is being evaluated by Massachusetts General Hospital. Pembrolizumab was developed by Merck Sharp & Dohme Corp., and is sold under the brand name Keytruda. | More Info | Learn more |
| M7824 | As a fusion protein (made from a fusion gene created by joining parts of two different genes), M7824 is designed to block two signaling pathways commonly used by cancer cells to avoid being destroyed by the immune system. M7824 consists of a monoclonal antibody against a protein called programmed death-ligand 1 (PD-L1) linked to the extracellular domain of the human transforming growth factor beta (TGF-β) receptor 2 protein. | Phase 2 | M7824 is being evaluated in RRP by the National Cancer Institute (NCI) | More Info | Learn more |
| Lenvatinib | Lenvatinib acts as a multiple kinase inhibitor. It inhibits the three main vascular endothelial growth factor receptors VEGFR1, 2 and 3, as well as fibroblast growth factor receptors (FGFR) 1, 2, 3 and 4, platelet-derived growth factor receptor (PDGFR) alpha, c-Kit, and the RET proto-oncogene. | Phase 2 | Lenvatinib (in combination with Pembrolizumab) is being evaluated by Massachusetts General Hospital. Lenvatinib was developed by Eisai, Inc., and is sold under the brand name Lenvima. | More Info | Learn more |
| INO-3107/ CELLECTRA™ 2000 | INOVIO's DNA medicines deliver optimized plasmids directly into cells intramuscularly or intradermally using INOVIO's proprietary hand-held smart device called CELLECTRA®. The CELLECTRA device uses a brief electrical pulse to reversibly open small pores in the cell to allow the plasmids to enter, overcoming a key limitation of other DNA and other nucleic acid approaches, such as mRNA. Once inside the cell, the DNA plasmids enable the cell to produce the targeted antigen. The antigen is processed naturally in the cell and triggers the desired T cell and antibody-mediated immune responses. Administration with the CELLECTRA device ensures that the DNA medicine is efficiently delivered directly into the body's cells, where it can go to work to drive an immune response. INOVIO's DNA medicines do not interfere with or change in any way an individual's own DNA. The advantages of INOVIO's DNA medicine platform are how fast DNA medicines can be designed and manufactured; the stability of the products, which do not require freezing in storage and transport; and the robust immune response, safety profile, and tolerability that have been demonstrated in clinical trials. | Phase 1/2 | Inovio Pharmaceuticals | More Info | Learn more |
† National Institute on Deafness and Other Communication Disorders (NIDCD)