Relapsing Polychondritis

What is Relapsing Polychondritis?

Relapsing polychondritis is a rare autoimmune condition with recurrent episodes of inflammation in cartilage, mainly in the ears, nose and windpipe.^{[1, 2]} It can also affect other tissues, including the eyes, joints and heart, so its effects are not limited to cartilage.^[3] It needs care from specialists such as a rheumatologist.

Common symptoms

The most typical features are inflammation of the ear and nose cartilage, joint pain, eye inflammation and involvement of the bronchial tubes.^{[3, 4]} Because symptoms are varied and nonspecific at the start, diagnosis is often delayed.^[4]

• Painful, red, swollen ears, and later deformity or stenosis of the ear canal
• Pain or a flattened bridge of the nose (saddle nose deformity)
• Joint pain and swelling
• Red or painful eyes
• Hoarseness, cough, wheezing or noisy breathing when the airways are involved

Airway involvement affects 20% to 50% of patients and can be the first sign of disease.^[5]

Causes and risk factors

The cause is not fully known. It is classed as an autoimmune or immune-mediated disease.^{[3, 4]}

It usually begins in middle-aged adults.^[6] Researchers have recently described three distinct groups of patients, with different features and outlooks, and some people have an associated blood disorder called VEXAS syndrome.^{[2, 6]}

How it is diagnosed and treated conventionally

There is no specific laboratory test, so diagnosis rests on clinical findings and on ruling out other conditions.^[6] Because the airways can be involved even without symptoms, doctors check them with chest CT and breathing tests at diagnosis and during follow-up.^[5]

Management is not standardized and depends on the person's symptoms. Mild forms may be treated with anti-inflammatory medicines or a short course of corticosteroids, sometimes with colchicine, while more serious disease uses the lowest possible steroid dose plus immune-suppressing drugs or targeted therapies.^[6] No randomized trial has been done because the disease is rare. In a systematic review of 177 patients, TNF inhibitors, tocilizumab and abatacept were linked to the best response rates.^[7]

Why Relapsing Polychondritis calls for a whole-person approach

Relapsing polychondritis comes in flares and quiet periods, and outcomes differ greatly between people. Forms limited to the ear or nose cartilage have a good outlook, while airway, heart and blood-disorder involvement make the outlook worse.^[6]

Living with unpredictable flares, long-term medicines and visible changes in the ears or nose affects more than the cartilage.

Immune-mediated inflammation

The disease is immune-mediated and recurs in relapses with periods of remission that can be very long.^{[4, 6]} This pattern is why specialist treatment aims to suppress inflammation over the long term.

Airway and heart involvement

Involvement of the bronchial tree is a leading cause of illness and death in this disease.^[3] Any new breathing symptom needs prompt medical assessment rather than supportive care.^[5]

Pain and joint symptoms

Joint pain and painful ear or nose flares are among the most common features.^[4] Chronic pain is one of the problems for which acupuncture has been studied in other conditions.^[8]

Fatigue, sleep and stress

Flares, unpredictable relapses and long-term medicines can drain energy and disturb sleep and mood. Fatigue and sleep are measurable symptoms for which supportive therapies have been studied in other conditions.^{[9, 10]}

Acupuncture as supportive care in Relapsing Polychondritis

What the research shows

No clinical trials of acupuncture for relapsing polychondritis were found. Evidence from other conditions gives only an indirect guide.

A pooled analysis of 39 trials with 20,827 patients found acupuncture better than sham and no acupuncture for chronic musculoskeletal pain, osteoarthritis and headache.^[8] For rheumatoid arthritis, an inflammatory joint disease, findings are mixed. A review of 7 systematic reviews concluded that acupuncture probably has little or no impact on the disease,^[11] while a network meta-analysis of 32 trials suggested some acupuncture-related therapies added to standard drugs may improve pain scores and disease activity.^[12]

For symptoms, a review of 24 trials in insomnia included a meta-analysis of 15 trials in which acupuncture improved sleep scores compared with medication, mostly after three or more weeks, with highly variable results.^[10] A review of acupuncture for chronic fatigue syndrome reported a benefit over sham with low-certainty evidence, but most studies were of low quality and no firm conclusion could be reached.^[9] None of this shows that acupuncture works for relapsing polychondritis.

How acupuncture may work

How acupuncture might act in relapsing polychondritis has not been studied. We do not claim that it affects the immune attack on cartilage or prevents flares.

What treatment involves

Treatment uses fine, sterile, single-use needles placed at selected points, usually left in for about 20 to 40 minutes. A course is typically several visits, with reassessment to see whether pain, sleep or fatigue is changing. During an ear or nose flare, we would avoid needling inflamed cartilage and would choose points elsewhere.

In prospective studies, about 9 in 100 patients had a minor reaction such as brief bleeding, soreness or redness at a needle site, and serious events occurred in about 1 in 10,000 patients.^[13] Tell the practitioner first if you take steroids or other immune-suppressing medicines, take blood thinners or have a bleeding disorder, have a heart valve problem, are pregnant, or have a pacemaker (relevant to electroacupuncture). Because immune-suppressing treatment can lower resistance to infection, we ask that your physician knows you are having needle treatment.

Ayurvedic medicine as supportive care in Relapsing Polychondritis

The Ayurvedic view

Ayurveda has no classical name for relapsing polychondritis. Practitioners may describe recurring inflammation and painful, deforming changes in terms of imbalance of the doshas, especially Vata and Pitta, and of ama (poorly processed material) in the tissues. These are traditional explanatory frameworks, not biomedical diagnoses.

Therapies that may be used

For supportive care, an Ayurvedic practitioner may suggest:

• Regular meals and a steady daily routine to support sleep and energy
• Gentle breathing practices and relaxation, only if your airways are not involved or your specialist agrees
• Warm oil massage for muscle and joint stiffness, avoiding inflamed ears or nose
• Individually chosen herbs, only after review of your medicines

Panchakarma is not appropriate during pregnancy, acute illness or frailty. It is also not suitable during a flare, with airway involvement or on high-dose immune suppression.

What the research shows

No clinical studies of Ayurvedic treatment for relapsing polychondritis were found, so there is no clinical evidence. Product quality is a real concern, because about one fifth of Ayurvedic medicines bought online contained detectable lead, mercury or arsenic.^[14] People taking immune-suppressing drugs need extra care with any herbal preparation.^[15]

Herbal medicine as supportive care in Relapsing Polychondritis

Herbs and formulas that have been studied

No herbal formula has been tested in a clinical trial for relapsing polychondritis. Traditional practice may use herbs for pain, heat and inflammation patterns, chosen individually by a qualified practitioner. We found no studies of herbs in people with this disease.

What the research shows

There is no clinical evidence. A single integrated Chinese and Western medicine case report has been published, but it has no abstract and cannot show benefit. Herbs are not a substitute for steroids, immune-suppressing drugs or biologic medicines, which are the treatments with the best reported response rates.^[7]

Safety and herb-drug interactions

Safety matters especially because many people with this condition take steroids, methotrexate, azathioprine, tocilizumab or other immune-modifying drugs. A review of herb-drug interactions describes St. John's wort lowering blood levels of immune-suppressing drugs such as ciclosporin and tacrolimus, and a systematic review lists interactions with a number of other prescribed drugs.^{[15, 16]} Herbal and dietary supplements account for about 20% of drug-induced liver injury cases in the United States, which is relevant for people taking methotrexate or azathioprine.^[17]

Herbs and Ayurvedic products should come from tested sources and be prescribed individually.^[14] Tell us if you are pregnant or breastfeeding, or have liver or kidney disease.

Translating traditional medicine into modern biological language

This is interpretation, not equivalence. Chinese medicine may describe recurring heat, swelling and pain in the ears or nose as heat toxin or blood stasis patterns, and Ayurveda may describe it as aggravated Pitta or Vata with ama. Researchers describe related ideas such as autoimmune inflammation of cartilage and relapse-remission cycles.^{[3, 6]} No study has shown that the traditional patterns correspond to these processes.

The realistic overlap is at the symptom level, such as pain, sleep and stress, which are measured with questionnaires in studies of other conditions.

How this care fits with your medical care

Complementary care for relapsing polychondritis is supportive only and begins after the condition is under specialist management. For a condition like this, we ask that you are under a physician's care for it and that your treating team knows you are receiving complementary care.

Do not stop or change prescribed medicines, including steroids, without your prescriber. We do not diagnose or monitor relapsing polychondritis. Please bring your diagnosis, recent test and airway or imaging results, a full list of medicines and supplements, and the names of your treating physicians. Keep your follow-up visits, since the airways and heart need regular checks.^[5]

When to seek urgent medical care

Relapsing polychondritis can affect the airway, eyes and heart, and some changes need prompt medical attention.^{[3, 5]}

• New or worsening shortness of breath, noisy or high-pitched breathing (stridor), or a change in voice
• Chest pain, palpitations or fainting
• Painful red eye, vision changes or sudden hearing loss
• Fever with a flare of ear, nose or joint inflammation
• Sudden severe pain, swelling or redness of the ear or nose that is rapidly worsening
• Fever or signs of infection while taking steroids or other immune-suppressing medicines

If any of these occur, call 911 or go to the nearest emergency department.