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PubMed This is a summary of 18 peer-reviewed journal articles Updated
Rheumatology

Managing Airway and Organ Involvement

At a Glance

Relapsing polychondritis can cause lasting airway narrowing or collapse and damage the aorta, heart valves, eyes, or nasal cartilage. When medicines are not enough, specialized procedures may help, but timing, disease control, and expert rescue planning are essential.

While medications are the first line of defense in relapsing polychondritis (RP), they cannot always fix the structural damage left behind by inflammation. When cartilage becomes too soft or the heart’s large vessels become weakened, specialized procedures and surgeries may be considered [1][2][3].

Managing Structural Airway Issues

If the cartilage in your windpipe (trachea) or the tubes leading to your lungs (bronchi) becomes too damaged, it can lead to two main issues: stenosis (a permanent narrowing) or tracheobronchomalacia (the airway becomes “floppy” and collapses when you breathe out) [4][5].

When medications aren’t enough to keep the airway open, several interventions may be considered at highly experienced airway centers:

  • Airway Stenting: A small tube (stent) made of silicone or metal is placed inside the airway to hold it open [6]. Stents are not routine first-line solutions in RP. They require frequent monitoring and carry risks of migration, infection, mucus buildup, or the growth of extra scar tissue called granulation tissue, which may complicate removal later [6][7].
  • Tracheostomy: In severe upper airway blockages, a surgeon may create a small opening in the neck to allow you to breathe directly through a tube [8]. However, a tracheostomy does not correct collapse or narrowing below the tube itself.
  • Balloon Dilation: For some types of fixed narrowing, a doctor can use a small balloon to gently stretch the airway open, though this may require repeat procedures [9][5].

Expert Caution: Any procedure involving your airway—even a simple look with a camera (bronchoscopy)—must be done by a specialized team with a rescue plan in place. In exceptional circumstances at tertiary centers, advanced support like ECMO might be used as a bridge, but this is highly specialized and not a standard expectation [10][11].

Cardiovascular and Heart Surgery

RP can weaken the aorta (the body’s main highway for blood) or damage the heart valves [12]. If an aortic aneurysm (a bulge) or a severe valve leak is identified, management depends on anatomy, size, growth, and symptoms [13][2].

Standard procedures include replacing sections of the aorta with a synthetic graft or replacing damaged heart valves [13][12]. Because the surrounding tissues can be fragile due to inflammation, planned surgeries are coordinated closely with your rheumatologist to ensure your immune system is quiet before the operation, whereas emergency procedures (like for a dissection) cannot wait [14][2].

Protecting Your Vision

Severe eye inflammation, such as necrotizing scleritis, is an emergency that threatens your vision and requires urgent care from an ophthalmologist and rapid systemic treatment [3]. Biologics like Tocilizumab have shown success in some of the most difficult eye cases in case series, but response is variable, and the specific medication must be tailored to the individual [15][3].

Reconstructive Surgery for the Nose

A “saddle-nose” deformity occurs when the nasal cartilage collapses. While reconstructive surgery (rhinoplasty) can restore the shape using cartilage grafts, it is not a decision to be made lightly [16][17].

The most important rule for nasal surgery in RP is timing. Most experts recommend that the surgeon and rheumatologist agree the disease is in stable remission before surgery is considered, though urgent functional surgery may have different considerations [16]. If the disease flares up again, your immune system may attack and damage the new cartilage graft, leading to graft resorption [16][18].

Common questions in this guide

How can relapsing polychondritis damage the airway?
Inflammation can weaken or scar the windpipe and the larger breathing tubes, causing either a fixed narrowing or an airway that collapses when you breathe out. These problems may persist even after inflammation is treated, so medicines alone may not always keep the airway open.
When might an airway stent or another airway procedure be used for RP?
A stent, balloon stretching, or a tracheostomy may be considered when medicines do not keep a damaged airway open, and decisions should be made at an experienced airway center. Stents are not routine first-line treatment and can move, become infected, collect mucus, or cause extra scar-like tissue, so they need close monitoring.
What heart problems from relapsing polychondritis may need surgery?
If RP weakens the aorta and causes an aneurysm, or damages a valve enough to cause a severe leak, surgery may be needed. Options include replacing part of the aorta with a synthetic graft or replacing the valve, based on the anatomy, size, growth, and symptoms.
Why does disease remission matter before nasal reconstruction?
Nasal reconstruction is usually planned when RP has been stable and quiet, because a flare can attack the cartilage graft and cause it to be absorbed. Your surgeon and rheumatologist should weigh the expected benefit against the possibility that the repair may not last.
What should I do if RP causes severe eye inflammation?
Severe inflammation of the white outer layer of the eye, called necrotizing scleritis, can threaten vision and needs urgent ophthalmology care and rapid treatment throughout the body. Medicines such as tocilizumab may help some difficult cases, but response varies and treatment must be individualized.
What safety planning is needed for bronchoscopy or airway surgery in RP?
Even bronchoscopy, which uses a camera to look inside the airway, can be risky when the airway is fragile. A specialized team should plan how to keep the airway open and respond to collapse; in rare, highly specialized situations, temporary heart-lung support called ECMO may be used.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Do I have fixed airway narrowing (stenosis) or floppy airways (malacia), and how does that change which procedure you recommend?
  2. 2.If we use a stent, which type (silicone or metallic) is safer for my specific anatomy, and how will we monitor for mucus buildup or new tissue growth (granulation)?
  3. 3.Is my disease 'quiet' enough right now to consider a planned heart or nasal procedure without risking a new flare or poor healing?
  4. 4.What is the 'rescue plan' if my airway becomes unstable during a planned surgery or bronchoscopy?
  5. 5.How will you and my ophthalmologist coordinate my medications if my eye inflammation becomes vision-threatening?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (18)
  1. 1

    Tracheobronchial involvement of relapsing polychondritis.

    de Montmollin N, Dusser D, Lorut C, et al.

    Autoimmunity reviews 2019; (18(9)):102353 doi:10.1016/j.autrev.2019.102353.

    PMID: 31323366
  2. 2

    Aortic involvement in relapsing polychondritis: case-based review.

    Erdogan M, Esatoglu SN, Hatemi G, Hamuryudan V

    Rheumatology international 2021; (41(4)):827-837 doi:10.1007/s00296-019-04468-5.

    PMID: 31768631
  3. 3

    Scleritis associated with relapsing polychondritis.

    Sainz-de-la-Maza M, Molina N, Gonzalez-Gonzalez LA, et al.

    The British journal of ophthalmology 2016; (100(9)):1290-4 doi:10.1136/bjophthalmol-2015-306902.

    PMID: 26888976
  4. 4

    Case of paediatric relapsing polychondritis with severe airway involvement: the challenges of long-term airway and respiratory management.

    Kothari T, Valsamakis T, Sridhar AV, Ahmed MI

    BMJ case reports 2021; (14(8)) doi:10.1136/bcr-2020-239774.

    PMID: 34380670
  5. 5

    Clinical diagnosis and treatment of pediatric-onset relapsing polychondritis with airway involvement.

    Wang Y, Xie Z, Chen J, Li X

    Frontiers in pediatrics 2025; (13()):1548142 doi:10.3389/fped.2025.1548142.

    PMID: 40438781
  6. 6

    Long-Term Outcome of Metallic Stenting for Central Airway Involvement in Relapsing Polychondritis.

    Wu X, Zhang X, Zhang W, et al.

    The Annals of thoracic surgery 2019; (108(3)):897-904 doi:10.1016/j.athoracsur.2019.02.039.

    PMID: 30910657
  7. 7

    Evaluation of airway involvement and treatment in patients with relapsing polychondritis.

    Handa H, Ooka S, Shimizu J, et al.

    Scientific reports 2023; (13(1)):8307 doi:10.1038/s41598-023-35616-4.

    PMID: 37221366
  8. 8

    Characteristics and Clinical Outcomes of 295 Patients With Relapsing Polychondritis.

    Chen N, Zheng Y

    The Journal of rheumatology 2021; (48(12)):1876-1882 doi:10.3899/jrheum.210062.

    PMID: 34334365
  9. 9

    [Diagnosis and treatment of laryngotracheal stenosis induced by relapsing polychondritis].

    Guo Z, Zhao D, Xing Y, et al.

    Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery 2020; (34(6)):524-527 doi:10.13201/j.issn.2096-7993.2020.06.011.

    PMID: 32842184
  10. 10

    Risk factors associated with severe adverse events in patients with relapsing polychondritis undergoing flexible bronchoscopy.

    Wang ST, Wang J, Gao X, et al.

    Orphanet journal of rare diseases 2024; (19(1)):54 doi:10.1186/s13023-024-03061-9.

    PMID: 38336719
  11. 11

    Bronchoscopy-Guided Intervention Therapy With Extracorporeal Membrane Oxygenation Support for Relapsing Polychondritis With Severe Tracheobronchomalacia: A Case Report and Literature Review.

    Zhou P, Fu B, Zhang C, et al.

    Frontiers in medicine 2021; (8()):695505 doi:10.3389/fmed.2021.695505.

    PMID: 34888317
  12. 12

    Concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting for aortic stenosis and mitral regurgitation in a patient with relapsing polychondritis.

    Kisamori E, Otani S, Yamamoto T, et al.

    General thoracic and cardiovascular surgery 2020; (68(2)):185-189 doi:10.1007/s11748-019-01130-6.

    PMID: 31041726
  13. 13

    [Rapid Growing Thoracic Aortic Aneurysm in a Patient with Relapsing Polychondritis].

    Yamamoto T, Adachi K, Hori D, et al.

    Kyobu geka. The Japanese journal of thoracic surgery 2021; (74(8)):583-586.

    PMID: 34334598
  14. 14

    Large-vessel Vasculitis Affecting the Aorta and its Branches in Relapsing Polychondritis: Case Series and Systematic Review of the Literature.

    Tomelleri A, Campochiaro C, Sartorelli S, et al.

    The Journal of rheumatology 2020; (47(12)):1780-1784 doi:10.3899/jrheum.190862.

    PMID: 31839593
  15. 15

    Sustained Remission with Tocilizumab in Refractory Relapsing Polychondritis with Ocular Involvement: A Case Series.

    Farhat R, Clavel G, Villeneuve D, et al.

    Ocular immunology and inflammation 2021; (29(1)):9-13 doi:10.1080/09273948.2020.1763405.

    PMID: 32643976
  16. 16

    Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis.

    Lee Y, Choi H

    Archives of craniofacial surgery 2019; (20(5)):341-344 doi:10.7181/acfs.2019.00437.

    PMID: 31658802
  17. 17

    Reconstructive rhinoplasty using cadaver cartilage in relapsing polychondritis.

    Shah R, Alford EL

    Proceedings (Baylor University. Medical Center) 2023; (36(1)):130-131 doi:10.1080/08998280.2022.2114067.

    PMID: 36578604
  18. 18

    Indications and Long-term Outcomes of Open Augmentation Rhinoplasty with Autogenous L-shaped Costal Cartilage Strut Grafts - A Single Plastic Surgeon's Experience.

    Leach L, Shamil E, Malata CM

    Otolaryngologia polska = The Polish otolaryngology 2018; (72(3)):26-32 doi:10.5604/01.3001.0011.7258.

    PMID: 29989560

This page is for informational purposes only and does not replace medical advice. Decisions about airway, heart, eye, or nasal procedures for relapsing polychondritis should be made with your rheumatologist and the appropriate specialists.

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