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PubMed This is a summary of 81 peer-reviewed journal articles Updated

Relapsing polychondritis: A Patient Guide

At a Glance

Relapsing polychondritis is a rare autoimmune disease that repeatedly inflames cartilage. Care focuses on quickly controlling inflammation, checking for hidden airway and aortic involvement, using steroid-sparing medicines when appropriate, and tailoring monitoring to each patient.

Relapsing polychondritis (RP) is a rare and complex autoimmune condition characterized by recurrent inflammation of the body’s cartilage. In a healthy body, cartilage provides essential structure and flexibility; however, in RP, the immune system mistakenly identifies this tissue as a foreign threat and launches an inflammatory attack. While this often first appears as painful swelling or redness in the “visible” cartilage of the ears and nose, RP is a systemic disease that can involve any tissue where specific types of collagen and proteins are present [1].

This guide is an educational resource. RP is rare, and much of the medical evidence is observational. Emergency symptoms always override routine plans, and you should never change immunosuppressive medications without your clinician.

The true challenge of living with RP lies in its ability to affect vital internal structures that you cannot see or feel. Beyond the ears and nose, the disease can target the cartilage holding your windpipe open, the valves within your heart, the aorta, and the delicate tissues of your eyes. Because damage to the airway or the aorta can progress “silently”—meaning it may cause no obvious symptoms until the damage is significant—specialist-guided baseline screening and individualized ongoing monitoring are important considerations for safe management [2][3].

Treatment for RP is highly individualized, as no two patients experience the same disease course. The primary goal of your medical team will be to rapidly suppress active inflammation to prevent permanent structural damage. While corticosteroids are often used to achieve this quickly, the long-term strategy focuses on introducing “steroid-sparing” immunosuppressants and biologics. These medications are used to keep the disease in remission while minimizing the side effects associated with long-term steroid use [4][5].

Recent scientific breakthroughs have further refined our understanding of this condition, particularly through the discovery of VEXAS syndrome. This condition, caused by an acquired genetic mutation, acts as a mimic or overlap syndrome. While not everyone with RP needs testing, evaluating for VEXAS is an important consideration for a specific subset of patients (typically older men) who experience severe symptoms that resist standard treatments. Ultimately, while an RP diagnosis brings uncertainty, a proactive approach centered on expert coordination and individualized monitoring provides the best path toward protecting your health and maintaining your quality of life [6][7].

Common questions in this guide

What is relapsing polychondritis, and which parts of the body can it affect?
Relapsing polychondritis is a rare autoimmune disease that causes repeated inflammation of cartilage and related tissues. It often affects the ears and nose, but it can also involve the airways, eyes, heart valves, and aorta.
Can relapsing polychondritis affect the airway or aorta without obvious symptoms?
Yes. Inflammation or damage involving the airway or aorta may progress with few noticeable symptoms until it is advanced, which is why clinicians may recommend individualized baseline screening and follow-up monitoring.
What are the treatment goals for relapsing polychondritis?
The first goal is to quickly control active inflammation and prevent lasting structural damage. Corticosteroids may provide rapid control, while steroid-sparing immune medicines and biologic medicines may be used longer term to help maintain remission and reduce steroid side effects.
Does everyone with relapsing polychondritis need VEXAS or UBA1 testing?
No. VEXAS syndrome can mimic or overlap with relapsing polychondritis, and clinicians may consider testing for its acquired UBA1 mutation in older men with severe symptoms that do not respond to standard treatment.
What symptoms should prompt urgent contact with my care team?
A sudden change in breathing can signal a serious airway problem, and a sudden change in vision needs prompt medical attention. Emergency symptoms take priority over routine appointments, so seek urgent or emergency care according to the severity of the change.
How is monitoring tailored for someone with relapsing polychondritis?
Because the disease course differs from person to person, clinicians use symptoms, individual risks, and treatment response to choose screening and follow-up. Monitoring may focus on hidden airway or aortic involvement as well as medication effects.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Does my current symptom pattern suggest that my internal organs, like my heart or lungs, are already involved?
  2. 2.What is the immediate plan for my baseline screening to check for 'silent' airway or aortic issues based on my risks?
  3. 3.Given my age and symptoms, should I be evaluated for the UBA1 mutation associated with VEXAS syndrome?
  4. 4.How will we decide which 'steroid-sparing' medication is the right first step for me?
  5. 5.Which specialist on my team should I contact first if I notice a sudden change in my breathing or vision?

Questions For You

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References

References (7)
  1. 1

    French practical guidelines for the diagnosis and management of relapsing polychondritis.

    Arnaud L, Costedoat-Chalumeau N, Mathian A, et al.

    La Revue de medecine interne 2023; (44(6)):282-294 doi:10.1016/j.revmed.2023.05.005.

    PMID: 37236870
  2. 2

    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
  3. 3

    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
  4. 4

    Relapsing polychondritis: A 2016 update on clinical features, diagnostic tools, treatment and biological drug use.

    Mathian A, Miyara M, Cohen-Aubart F, et al.

    Best practice & research. Clinical rheumatology 2016; (30(2)):316-333 doi:10.1016/j.berh.2016.08.001.

    PMID: 27886803
  5. 5

    Biologic therapy in relapsing polychondritis: navigating between options.

    Padoan R, Campaniello D, Iorio L, et al.

    Expert opinion on biological therapy 2022; (22(5)):661-671 doi:10.1080/14712598.2022.2048647.

    PMID: 35230215
  6. 6

    Somatic Mutations in UBA1 Define a Distinct Subset of Relapsing Polychondritis Patients With VEXAS.

    Ferrada MA, Sikora KA, Luo Y, et al.

    Arthritis & rheumatology (Hoboken, N.J.) 2021; (73(10)):1886-1895 doi:10.1002/art.41743.

    PMID: 33779074
  7. 7

    VEXAS: where do we stand 2 years later?

    Sujobert P, Heiblig M, Jamilloux Y

    Current opinion in hematology 2023; (30(2)):64-69 doi:10.1097/MOH.0000000000000750.

    PMID: 36728604

This guide about relapsing polychondritis is for informational purposes only and does not constitute medical advice. Do not change immunosuppressive medicines or delay urgent care without guidance from your clinician.

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