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Relapsing Polychondritis, Causes, Diagnosis, and Treatments

Updated: Mar 12

Do You suffer from Painful Ears, Nose, Eyes, Joints? Are you Wondering if You Might Be Suffering from Relapsing Polychondritis?

Read on to Learn about Relapsing polychondritis, causes and treatments.


What is Relapsing Polychondritis

Relapsing polychondritis is a rare, incurable autoimmune disease with an unknown cause and an estimated incidence between .71 to 3.5 per million per year.

It is a connective tissue disorder characterized by recurrent progressively destructive episodes of extremely painful inflammation of cartilage throughout the body (ears, nose, larynx, trachea, eyes, joints, kidney, or heart) resulting in progressive anatomical deformation and loss of function.

It can be debilitating (“cauliflower” ears, saddle-nose, blindness, deafness…) and life-threatening, particularly when it moves into the respiratory system.

RP is seen with equal frequency in both sexes and all racial groups, with a slightly higher preponderance in women.

The median age of onset is between 40 and 50 years of age, however it can occur at any age. About 25% of people with autoimmune diseases have a tendency to develop additional autoimmune diseases.

In about 30% of cases the RP is a secondary autoimmune disorder, accompanied by other systemic connective tissue disorders such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjögren’s syndrome, thyroiditis, ulcerative colitis, psoriasis and Behçet’s syndrome.

Because of the remitting and variable nature of RP and due to the non-specific symptoms at the onset (fever, weight loss, night sweats, fatigue, lymph node enlargement), the diagnosis is often delayed, on average by about 2.9 years. [1]


How Is Relapsing Polychondritis Diagnosed

There is no specific blood test for RP, the disease is diagnosed clinically but biopsy and imaging are sometimes used.

It often takes years before rare autoimmune diseases get diagnosed.


Doctor telling patient there is nothing wrong with her
Doctor telling patient that there is nothing wrong with her


An RP diagnosis is made when a patient meets 3 of the following 6 criteria:

  1. Recurrent chondritis of both auricles (up to 90% of patients)

  2. Nonerosive inflammatory arthritis (50–85% of patients)

  3. Chondritis of nasal cartilages (53% of patients)

  4. Inflammation of ocular structures: conjunctivitis/keratitis/scleritis/uveitis (50–60% of cases)

  5. Chondritis of respiratory tract: laryngeal, tracheal cartilage

  6. Cochlear or vestibular damage: neurosensory hearing loss/tinnitus/vertigo



Conventional Treatment of Relapsing Polychondritis

Due to the rarity of RP, there is no standardized treatment approach, and the treatment is guided by the clinical presentation and the severity of the disease.


Drugs for Treating Relapsing Polychondritis

Mild cases without serious organ damage are treated with glucocorticoids (e.g., prednisone) or dapsone.

In severe cases, other immunosuppressive drugs are used including methotrexate and cyclophosphamide.

Biologics have been used, most commonly TNF inhibitor-Infliximab. Other biologics like adalimumab, etanercept, abatacept, tocilizumab have been tried with variable results. The use of rituximab for RP was unsuccessful. [2,3]


Various pills
Various pills


Problem with Drugs

Drugs do not treat the true cause, they only mask the symptoms! Some people do not respond to some drugs, all drugs have side effects and are expensive.

Examples of common RP medication side effects: blurred vision, fast, slow, pounding, or irregular heartbeat or pulse, noisy, rattling breathing, numbness or tingling in the arms or legs, trouble thinking, speaking, or walking, fluid retention, weight gain, cataracts and increased intraocular pressure/glaucoma, osteoporosis, vertebral compression fractures, bluish fingernails, lips, or skin, difficult breathing, deadly infections, tuberculosis, blood in the urine or stools, bloody vomit, diarrhea, joint pain, reddening of the skin, sores in the mouth or lips, stomach pain, swelling of the feet or lower legs, hair loss (temporary), loss of appetite, nausea or vomiting, acne, boils on skin, pale skin, skin rash, or itching, tender, swollen glands in the neck, tightness in the chest, trouble swallowing, troubled breathing with exertion, unusual bleeding or bruising, unusual tiredness or weakness…


Surgery for Relapsing Polychondritis

In case of airway collapse, surgical intervention such as stenting, airway dilation, tracheostomy and laryngotracheal reconstruction are performed.


The Cause of Relapsing Polychondritis

Since the etiology (cause) of RP is unknown, the treatment consists of reducing inflammation by suppressing the immune system via drugs or surgery. Not a single RP-specific research study goes deeper and looks into what causes this inflammation!


 

If you look up the term Relapsing Polychondritis (RP) in the National Library of Medicine, the largest medical library in the world, you will find close to 2,000 studies. None of them offers much hope or mentions a connection between RP and food. And yet, food might be the key to solving this medical mystery.


 


What Really Causes Relapsing Polychondritis?

I wanted to get to the bottom of what provokes such abnormal immune response that the body would turn against itself and start attacking cartilage. Luckily, there is plenty of research offering some clues.


 

According to Autoimmune Association, previously AARDA,

50 million Americans have an autoimmune disease.

 

There are over 100 autoimmune diseases​ and any body part can be involved.

 

Although each autoimmune disease has different symptoms,

they all are basically the same disease.

 

Inflammation, immune function dysregulation,

and loss of self-tolerance and the causes of thereof are common to all of them.

 

Key Variables That Play a Role in the Development of Autoimmune Diseases - Genetics & Environmental Factors (Especially Diet)!

The good news is that genes alone do not cause autoimmunity, it is the environmental factors what turns the genes on. In other words, genes load the gun, but environmental factors such as diet and lifestyle (which we have great power over) pull the trigger!


How Do You Develop Relapsing Polychondritis?

  • You have a genetic predisposition to RP.

  • Due to environmental factors, you develop barrier permeability (leaky gut, bacterial dysbiosis, leaky lungs, leaky brain, even leaky skin) leading to endotoxemia and increased antigen load.

  • When that happens, undigested food particles, viruses, bacteria and toxins “leak” into the body where they don’t belong and would under normal circumstances not get.

  • Your immune system starts producing inflammatory molecules and antibodies to destroy these foreign invaders (antigens).

  • When these antigens are similar enough to your own cartilage (or any other tissue in your body in the case of other autoimmune diseases), your immune system gets confused and once your body stops recognizing itself, you lose immune tolerance. Your immune system starts reacting to your own tissues via a process called Molecular Mimicry.

  • Your confused immune system might also start reacting to any other antigen (food, environmental compounds and pathogens).

  • Besides Molecular Mimicry, there are other mechanisms that lead to autoimmunity. Some of them are:

    • An insufficient number of T-regulatory cells that under normal circumstances turn off the immune attack when all antigens have been destroyed.

    • Too many natural killer cells (NKC) and cytotoxic T-cells are deployed to kill the antigen.

    • Overzealous B-cells accidentally mark innocent bystanders as foreign invaders.

    • Parasitic infections and food intolerances can lead to the overproduction of B-cells.

    • A chronic virus infection can lead to the overproduction of NKC and cytotoxic T-cells…


 

It is clear that we cannot do much to change our genes. We can, however, do a lot to

prevent Leaky Gut, which plays a major role in the development of autoimmunity,

by avoiding exposure to harmful, inflammatory environmental substances.

Impaired intestinal barrier function aka Leaky Gut often plays a key role

in the development of autoimmunity.

 

How Do You Develop a Leaky Gut? – Quite easily, actually.

Environmental factors that play a role in the development of leaky gut:

  • Poor diet (dairy, gluten, animal foods, highly processed foods, chlorinated water, excessive alcohol consumption)

  • Gut dysbiosis (an overgrowth of pathogenic bacteria)

  • Medications (antibiotics, NSAIDs, antacids, contraceptives, steroids, chemotherapy drugs, radiation therapy)

  • Chronic inflammation, infections and stress

  • Hormone imbalances

  • Poor GI function (constipation or diarrhea)

  • Intestinal inflammation

  • Neurological conditions (brain trauma, stroke, neurodegeneration)


Healing Leaky Gut

Luckily, once we stop the injury and fix the dysbiosis, over several weeks the gut will heal. The immune system will calm down and within weeks or months, flare-ups might become less frequent, less severe and in some cases might disappear altogether.


 

It is imperative that you discover what triggers your flares (food/environmental factors/stress/insufficient sleep) and avoid these triggers!

 


Where Does this Healthy, Healing, Anti-inflammatory Diet Come from?

As mentioned above, my search for scientific studies concentrating specifically on the connection between RP and diet was unsuccessful.

My diet recommendations are based on several studies that looked into diet and arthritis/lupus. Since RP shares many similarities with these diseases, similar strategies should work for reversing RP as well. (They worked for me and many others. You have nothing to lose by trying the diet, in fact you have a lot to gain.)

A number of these studies show that dietary changes can help relieve symptoms or even put autoimmune disease into remission. And since a healthy diet does not have any bad side effects and is of no additional cost (you have to eat anyway), it seems like the best strategy we have to conquer RP and other autoimmune diseases.


I promised to give you information about relapsing polychondritis, causes and treatments. Now that we learned about the causes of relapsing polychondritis, let's dive into the studies offering ideas for diet treatments.


Diet Studies

  • A 2013 systematic review looked into the associations between dietary patterns and inflammatory biomarkers and concluded that meat-based “Western” diet patterns are inflammatory whereas vegetable- and fruit-based or “healthy” patterns tend to be inversely associated. [6]


  • A review of clinical trials and observational studies found strong and consistent evidence that a plant-based dietary pattern can reduce inflammation, joint swelling, and BMI and improve gut bacteria. [7]


  • A vegan diet free of gluten improves the signs and symptoms of rheumatoid arthritis. [8]


  • A very low-fat, vegan diet in patients with moderate-to-severe RA led to significant reductions in RA symptoms. [9]


  • Following a raw vegan diet rich in antioxidants and fiber (berries, fruits, vegetables and roots, nuts, germinated seeds and sprouts) can also decrease joint stiffness and pain in patients with RA. [10]


  • A systematic review concluded that fasting followed by a vegetarian or vegan diet might be useful in the treatment of RA. [11]


  • During a 2-week period of modified fast, joint pain in 20 patients with arthritis and various skin diseases improved in many subjects. Once a vegan diet (high in fat (42%) using vegetable oils) was introduced, RA symptoms returned in most, with the exception of some psoriasis patients. [12]


  • In a randomized, single-blind controlled trial, 27 patients spent four-weeks at a health farm. After an initial 7- to 10-day subtotal fast, they were put on a gluten-free vegan diet for 3.5 months. After four weeks at the health farm the diet group showed a significant improvement in RA symptoms. [13]


  • A 1994 clinical trial found significant beneficial changes in intestinal flora after patients shifted to a vegan diet for one year. [14]


  • Dr. Brooke Goldner, who cured herself from lupus, published a case series of two patients with SLE-related nephritis. By adopting a customized, raw, whole-food, plant-based eating regimen, high in leafy greens and cruciferous vegetables, omega-3 polyunsaturated fatty acids, and water for six weeks, her patients’ (female 24 and male 41) symptoms resolved, and laboratory tests were normalized. In addition, the female patient no longer needed dialysis or kidney transplant. [15]


  • A 2019 study published in the Journal of Translational Autoimmunity called Immunodietica: A data-driven approach to investigate interactions between diet and autoimmune disorders analyzed shared antigens between human tissue and common foods. The study concluded that pig shared the highest homology with human tissue antigen, followed by beef. The diseases that shared unique epitopes with pig were MS, RA, autoimmune disease of the eyes, ears, nose and throat, Behçet’s syndrome, and systemic autoimmune disease. The diseases that shared unique epitomes with cow were Guillain-Barré Syndrome, demyelinating polyneuropathy, RA, antiphospholipid syndrome, and vitiligo. An interactive website to explore this database has been created and is available at www.immunodietica.com. According to this database, RP shared non-unique epitopes (parts of an antigen that is recognized by the immune system, specifically by antibodies, B cells, or T cells) with American bison, cow, goat, rabbit, pig, sheep and other animal food samples. [16]

Conclusions:

  • To heal from Relapsing Polychondritis (or any other chronic disease), identify the cause (of inflammation) and treat the cause.

  • The identification and elimination of environmental and food triggers is crucial.

  • A whole-foods, high-raw, high-fiber, vegan, gluten-free, oil-free, sugar-free and salt-free diet has been proven to alleviate symptoms and even cure autoimmune disease.

  • By switching to a health-promoting diet, we change our gut microbiota, fix leaky gut, our immune system calms down, inflammation goes down and our bodies heal.

  • In severe cases, or to speed up the process of improving gut integrity, supplementing with probiotics, an elimination diet or a medically supervised water fast might be helpful.

  • Herbal remedies that lower inflammation such as turmeric [17], ginger [18] and cinnamon [19] are useful adjuvants, however it is the overall dietary pattern that will make a change.

  • Flares are almost always due to non-compliance on the diet and/or stress.

  • Flares will become less frequent and their strength less severe the longer patients stay on the diet.

  • Other lifestyle strategies that go beyond diet change such as not smoking, avoiding unnecessary vaccines, antibiotics and inflammation-causing medications, regular exercise, adequate sleep, maintaining healthy relationships, having a positive outlook on life, and stress reduction are also health-promoting and necessary to achieve optimal health.

  • In general, the goal in treating autoimmunity is to stop the progression of the autoimmune disease/s you currently have and possibly reverse it/them, but especially, to stop new autoimmune diseases from developing.


 

Having gone myself through an autoimmune diagnosis and the physical and emotional pain that comes with it I understand how it feels and my heart goes out to all autoimmune-disease sufferers out there. I made it my mission to help as many people with autoimmune disease as possible.


If you give me the privilege to guide you on your way to recovery,

I will be happy to assist you.



 


Resources:

[1] Borgia F, Giuffrida R, Guarneri F, et al. “Relapsing Polychondritis: An Updated Review.” Biomedicines. 2018 Sep; 6(3): 84. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6164217/ [2] Chauhan K, Surmachevska N; Hanna A. “Relapsing Polychondritis.” StatPearls Publishing; 2020 Jan [3] Alqanatish JT, Alfarhan BA, Qubaiban SM. “Limited auricular relapsing polychondritis in a child treated successfully with infliximab.” BMJ Case Rep. 2019; 12(5): e227043. [4] Martín R, Chamignon C , Mhedbi-Hajri N et al. “The potential probiotic Lactobacillus rhamnosus CNCM I-3690 strain protects the intestinal barrier by stimulating both mucus production and cytoprotective response.” Sci Rep. 2019; 9: 5398. [5] Serratrice J, Ené N, Granel B et al. “Severe Relapsing Polychondritis Occurring After Ear Piercing.” J Rheumatol. 2003 Dec;30(12):2716-7. [6] Barbaresko J, Koch M, Schulze MB et al. “Dietary pattern analysis and biomarkers of low-grade inflammation: a systematic literature review.” Nutr Rev. 2013 Aug;71(8):511-27. [7] Alwarith J, Kahleova H, Rembert E, et al. “Nutrition interventions in rheumatoid arthritis: The potential use of plant-based diets. A review.” Front Nutr. Published online September 10, 2019 [8] HafströmI, Ringertz B, Spångberg A. “A vegan diet free of gluten improves the signs and symptoms of rheumatoid arthritis: the effects on arthritis correlate with a reduction in antibodies to food antigens.” Rheumatology (Oxford). 2001 Oct;40(10):1175-9. [9] McDougallJ, Bruce B, Spiller G et al. “Effects of a very low-fat, vegan diet in subjects with rheumatoid arthritis.” J Altern Complement Med. 2002 Feb;8(1):71-5. [10] Hänninen, Kaartinen K, Rauma AL, et al. “Antioxidants in vegan diet and rheumatic disorders.” Toxicology. 2000;155:45-53. [11] Müller H, de Toledo FW, Resch KL. “Fasting followed by vegetarian diet in patients with rheumatoid arthritis: a systematic review.” Scand J Rheumatol. 2001;30:1-10. [12] Lithell H, Bruce A, Gustafsson IB, et al. “A fasting and vegetarian diet treatment trial on chronic inflammatory disorders.” Acta Derm Venereol. 1983;63:397-403. [13] Kjeldsen-KraghJ, Haugen M, Borchgrevink CF, et al. “Controlled trial of fasting and one-year vegetarian diet in rheumatoid arthritis.” Lancet. 1991 Oct 12;338(8772):899-902. [14] PeltonenR, Kjeldsen-Kragh J, Haugen M, et al. “Changes of faecal flora in rheumatoid arthritis during fasting and one-year vegetarian diet.” Br J Rheumatol. 1994 Jul;33(7):638-43. [15] Goldner B. “Six Week Raw Vegan Nutrition Protocol Rapidly Reverses Lupus Nephritis: A Case Series.” International Journal Of Disease Reversal And Prevention. Vol 1 No 1 (2019) [16] Gershteyn IM, M.R.Ferreira LMR. “Immunodietica: A data-driven approach to investigate interactions between diet and autoimmune disorders.” Journal of Translational Autoimmunity. Volume 1, April. [17]B. Chandran, A. Goel. “A randomized, pilot study to assess the efficacy and safety of curcumin in patients with active rheumatoid arthritis.” Phytother Res. 2012 26(11):1719 – 1725. [18] Aryaeian N, Mahdi Mahmoudi M, Shahram F et al. “The effect of ginger supplementation on IL2, TNFα, and IL1β cytokines gene expression levels in patients with active rheumatoid arthritis: A randomized controlled trial.” Med J Islam Repub Iran. 2019 Dec 27;33:154. [19] Shishehbor F, Safar MR, Rajaei E et al. “Cinnamon Consumption Improves Clinical Symptoms and Inflammatory Markers in Women With Rheumatoid Arthritis.” J Am Coll Nutr. 2018 May 3;1-6.

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