Relapsing polychondritis

Relapsing polychondritis is an autoimmune condition, which means the immune system mistakenly attacks the body's own tissues. Here the target is cartilage: the firm, flexible tissue that gives the ears and nose their shape, supports the voice box and windpipe, and cushions the joints. During a flare the immune system inflames the cartilage, and with repeated flares the cartilage can soften and lose its structure.

Why this happens is not fully understood. There is no single known trigger, and it is not infectious or passed directly from parent to child. The condition typically begins in middle age and affects both men and women. It can occur on its own, or alongside another autoimmune condition such as rheumatoid arthritis, lupus or vasculitis, so part of the assessment is looking carefully for any associated condition.

The condition typically comes and goes in flares, with better spells in between. Features may include:

• A painful, red, swollen ear that spares the soft earlobe, which has no cartilage. Repeated flares can soften the ear so it loses its firmness (sometimes called a floppy ear), and hearing and balance can be affected
• Tenderness over the bridge of the nose, and with repeated flares a change in the shape of the nose (a saddle-nose appearance)
• Hoarseness, cough, breathlessness or noisy breathing, when the cartilage of the voice box or windpipe is inflamed. This is the most serious form of involvement and the main reason for urgent care
• Joint pain or arthritis, which can move between joints
• Eye inflammation, causing red, painful eyes that may be sensitive to light
• Occasionally, involvement of the heart valves

Not everyone develops all of these, and in many people the ears and joints are the main features.

There is no single test for relapsing polychondritis. The diagnosis rests on recognising the characteristic pattern, such as repeated flares of cartilage inflammation in the ears or nose, often building up over months or years, together with a careful history and examination. Because the condition is rare and each flare can settle on its own, the diagnosis is frequently delayed, and many people have seen several doctors before the pattern is recognised.

Blood tests usually show general signs of inflammation, but there is no specific antibody that confirms the condition, so the tests support the picture rather than proving it. Occasionally a small biopsy of affected cartilage is taken to help secure the diagnosis. If there is any suggestion of airway involvement, scans of the voice box and windpipe and breathing tests are arranged, as this guides both the urgency and the shape of treatment.

Because their features overlap, part of a careful assessment, particularly in men over 50, is considering VEXAS syndrome. This is a condition first described in 2020 that can cause inflammation of the ear and nose cartilage closely resembling relapsing polychondritis. A helpful clue is an unexplained low blood count with enlarged red cells, known as a macrocytic anaemia, found alongside the inflammation. Where that pattern is present, a specific genetic test, looking for an acquired change in a gene called UBA1, can tell the two conditions apart. The distinction matters, because VEXAS is managed differently and needs specialist input. Our guide to VEXAS syndrome explains it in more detail.

Where joints are painful or swollen, ultrasound can help clarify whether inflammation is present. Dr Borukhson uses point-of-care ultrasound during the consultation. You can read more on the ultrasound clinic page.

Treatment is tailored to how active the condition is and which parts of the body are involved. Flares are usually settled with steroids, which calm cartilage inflammation quickly; milder ear or joint flares may need only modest doses, while more serious involvement is treated more intensively.

If flares keep returning, the aim shifts to preventing them rather than simply treating each one. Steroid-sparing immunosuppressant medicines, such as methotrexate or azathioprine, are used to keep the condition controlled while allowing the steroid dose to be reduced. In cases that do not respond, biologic medicines that target specific parts of the immune system are an option.

Involvement of the voice box or windpipe needs particular care. It is managed jointly with airway and other specialists as part of a multidisciplinary team, with monitoring of the airway alongside treatment of the inflammation. The overall aim of treatment is to settle flares promptly, protect the cartilage from lasting damage, and keep long-term medication to the minimum that holds the condition steady.

Between flares, a few consistent habits help keep the condition steady and catch problems early.

• Take your medicines as prescribed. If you are on steroids, never stop them suddenly; the dose is always reduced gradually under guidance.
• Learn your own flare pattern. Photographs of an inflamed ear or nose and a simple note of when flares happen are genuinely useful at review, and help treatment decisions.
• Reduce your infection risk while on immunosuppressants. Keep up with recommended vaccinations and raise any fever or infection promptly.
• Report changes in your voice, breathing, hearing or vision early. These are the areas where prompt treatment matters most.
• Attend follow-up appointments and blood tests. These keep the condition and the medicines safely monitored.

Many people with relapsing polychondritis live full, active lives, particularly when the condition is recognised early and flares are treated promptly.

Because relapsing polychondritis can involve the airway and the eyes, some symptoms need immediate attention rather than waiting for the next appointment.

Call 999 or go to A&E immediately if you develop:

• Noisy breathing, or a high-pitched sound when breathing in (stridor)
• Marked or rapidly worsening breathlessness
• A feeling that your throat or windpipe is closing

Seek same-day assessment for a new red, painful eye, particularly if it is sensitive to light or your vision is affected, as untreated eye inflammation can threaten sight.

Arrange a prompt review for a new flare of ear or nose pain and swelling, a new change in your voice or a persistent cough, new hearing loss or dizziness, or fever and feeling unwell while taking an immunosuppressant medicine.

Stable, settled symptoms and routine medication questions can wait for a planned appointment. When in doubt, it is always safer to be checked.

Relapsing polychondritis is rare, and because each flare can settle on its own the diagnosis is often delayed, sometimes by years. That delay matters: repeated untreated flares can permanently soften the cartilage of the ear or nose, and unrecognised airway involvement is the most serious risk of the condition. A careful specialist assessment can recognise the pattern, check for any associated autoimmune condition, arrange the right tests for the airways and eyes, and put in place a plan that treats flares promptly and works to prevent the next one. If you have had repeated episodes of a painful, swollen ear or nose, particularly with joint pain or eye inflammation, a considered rheumatology review is a sensible step.

Why does relapsing polychondritis affect the ear but spare the earlobe?

The condition attacks cartilage, and only the firm upper part of the outer ear contains cartilage. The soft earlobe has none, so it stays normal even when the rest of the ear is painful, red and swollen. This sparing of the lobe is a useful clue, because an infection of the ear tends to involve the whole ear, lobe included, whereas a flare of relapsing polychondritis characteristically stops where the cartilage ends.

How is relapsing polychondritis diagnosed?

There is no single test. The diagnosis rests on the characteristic clinical pattern, such as repeated flares of ear or nose cartilage inflammation, building up over time, together with a careful history and examination. Blood tests usually show general inflammation but there is no specific antibody for the condition, which is one reason the diagnosis is often delayed. Occasionally a small biopsy of cartilage is taken to support the diagnosis, and if the airways may be involved, scans and breathing tests are arranged.

What does treatment for relapsing polychondritis involve?

Flares are usually settled with steroids, which calm cartilage inflammation quickly. If flares keep returning, steroid-sparing immunosuppressant medicines such as methotrexate or azathioprine are used to keep the condition controlled while reducing the need for long-term steroids. Biologic medicines are an option in resistant cases. Where the voice box or windpipe is involved, care is shared with airway specialists as part of a multidisciplinary team, because this is the most serious form of the condition.

Is relapsing polychondritis linked to other autoimmune conditions?

It can be. Relapsing polychondritis sometimes occurs alongside another autoimmune condition, such as rheumatoid arthritis, lupus or vasculitis, and part of the specialist assessment is checking carefully for any associated condition. Where one is found, the treatment plan takes both conditions into account, and regular review helps keep each of them well controlled.

When should I seek urgent help with relapsing polychondritis?

Noisy breathing, a high-pitched sound when breathing in (stridor) or marked breathlessness can mean the cartilage of the voice box or windpipe is inflamed and narrowing the airway. This is an emergency: call 999 or go to A&E without delay. A new red, painful eye that is sensitive to light needs same-day assessment, as untreated eye inflammation can threaten sight. New ear or nose flares, or feeling unwell with a fever while on treatment, deserve a prompt review rather than waiting for a routine appointment.