Table of ContentsView AllTable of ContentsSymptomsComplicationsCausesDiagnosisTreatmentPrognosis
Table of ContentsView All
View All
Table of Contents
Symptoms
Complications
Causes
Diagnosis
Treatment
Prognosis
Relapsing polychondritis(RP) is a rareautoimmune disorderthat causes recurrentinflammationofcartilage. RP can affect any part of the body with cartilage, including the ears, nose, airways, ribcage, and joints. Symptoms occur in episodic flares, causing redness, swelling, pain, and other symptoms based on the organ or tissues affected.
Most cases of RP can be managed with medications such as over-the-counter painkillers and prescription steroids. Rare complications can be debilitating and even life-threatening.
This article describes the symptoms and causes of relapsing polychondritis. It also explains how RP is diagnosed and treated as well as the likely outcome (prognosis) of the rare autoimmune condition.
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What Are the Symptoms of Relapsing Polychondritis?
Relapsing polychondritis is so-named because it causes recurrent (relapsing) symptoms in many parts of the body. The prefixpoly-means “many,” while -chondritisrefers to the inflammation of cartilage cells (chondrocytes).
Possible Complications
While most cases of RP involve the ears, nose, or joints, the effects can be more severe if internal organs or adjacent tissue (like nerves) are affected. Some of these complications, while rare, can become life-threatening:
Overview of Perichondritis of the Ear
What Causes Relapsing Polychondritis?
The exact cause of relapsing polychondritis is unknown. RP is regarded as autoimmune because 50% of people with RP carry a gene called human leukocyte antigen DR4 (HLA-DR4). This is the same gene found in people with autoimmune diseases like lupus, multiple sclerosis (MS), rheumatoid arthritis (RA), type 1 diabetes, autoimmune hepatitis, and celiac disease (CD).
Even so, no specific autoantibody has yet been linked to RP. An autoantibody is an immune protein that targets and attacks specific cells in the body withautoimmune diseaseslike lupus or RA. RP can only be presumed to be autoimmune given the lack of this identifying feature.
Other environmental factors may contribute. but these are as of yet unidentified.
How Rare Is Relapsing Polychondritis?Relapsing polychondritis is rare, affecting 3.5 out of every 1,000,000 people each year. Most cases involve people in their 40s and 50s, but RP can affect anyone, even babies. Females are only slightly more affected than males. All races are equally at risk.
How Rare Is Relapsing Polychondritis?
Relapsing polychondritis is rare, affecting 3.5 out of every 1,000,000 people each year. Most cases involve people in their 40s and 50s, but RP can affect anyone, even babies. Females are only slightly more affected than males. All races are equally at risk.
How Is Relapsing Polychondritis Diagnosed?
There isn’t a specific test for making a diagnosis of relapsing polychondritis. Your healthcare provider will perform a physical exam and ask you about symptoms. They may request bloodwork to look for signs of inflammation or X-rays to see affected areas.
Diagnosis of RP involves meeting three of six specific criteria:
Depending on the symptoms you are experiencing, your healthcare provider may request abiopsy. This involves taking a small amount of tissue to look at under a microscope.
How Is Relapsing Polychondritis Treated?
There is no cure for relapsing polychondritis, but the condition is treatable. The main goals of treatment are to relieve symptoms and preserve cartilage in the affected area.
Options include:
Other DMARDs like Imuran (azathioprine), Cytoxan (cyclophosphamide), Dapsone (diaminodiphenyl sulfone), Arava (leflunomide), Myfortic (mycophenolate), and Neoral (cyclosporine) can be used with or without methotrexate if methotrexate is unable to provide sustained relief.
You may need to see several specialists to treat RP based on the symptoms you have. This may include arheumatologist(an expert in autoimmune diseases), acardiologist(a heart specialist), a nephrologist (a kidney specialist), and an ophthalmologist (an eye specialist), among others.
Overall, the outlook looks good. A 2016 study published inClinical Epidemiologysuggests that the five-year survival rate for RP is as high as 92%, meaning that 92 out of 100 people with RP will live forat leastfive years. The 10-year survival rate is nearly as good at 88%.
With that said, some complications carry a far higher risk of death. One such example is the development of kidney damage with RP. This complication is associated with a 10-year survival rate of only 10%.
Summary
Relapsing polychondritis (RP) is a rare autoimmune disease that causes the inflammation of cartilage throughout the body, particularly in the ears, nose, and joints. The symptoms and severity can vary from person to person. In rare instances, potentially life-threatening complications can develop involving the heart, airways, and kidneys.
Although there is no cure for relapsing polychondritis, it can be managed effectively with medications like prednisone and methotrexate.
5 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Genetic and Rare Diseases Information Center (GARD).Relapsing polychondritis.Borgia F, Giuffrida R, Guarneri F, et al.Relapsing polychondritis: an updated review.Biomedicines. 2018 Sep;6(3):84. doi:10.3390/biomedicines6030084Sosada B, Loza K, Bialo-Wojcicka E.Relapsing polychondritis.Case Rep Dermatol Med.2014; 2014:791951. doi:10.1155/2014/791951Lepka FK, Chevalier X.Refractory relapsing polychondritis: challenges and solutions.Open Access Rheumatol.2018;10:1–11. doi:10.2147/OARRR.S142892Horvath A, Pall N, Molnar K, et al.A nationwide study of the epidemiology of relapsing polychondritis.Clin Epidemiol.2016;8:211–230. doi:10.2147/CLEP.S91439Additional ReadingGulati A, Bagga A.Large vessel vasculitis.Pediatr Nephrol. 2010 Jun; 25(6):1037–1048. doi:10.1007/s00467-009-1312-9Lucerna A, Espinosa J.Acute atraumatic pinna (auricular) perichondritis.World J Emerg Med. 2018;9(2):152–153. doi:10.5847/wjem.j.1920-8642.2018.02.013
5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Genetic and Rare Diseases Information Center (GARD).Relapsing polychondritis.Borgia F, Giuffrida R, Guarneri F, et al.Relapsing polychondritis: an updated review.Biomedicines. 2018 Sep;6(3):84. doi:10.3390/biomedicines6030084Sosada B, Loza K, Bialo-Wojcicka E.Relapsing polychondritis.Case Rep Dermatol Med.2014; 2014:791951. doi:10.1155/2014/791951Lepka FK, Chevalier X.Refractory relapsing polychondritis: challenges and solutions.Open Access Rheumatol.2018;10:1–11. doi:10.2147/OARRR.S142892Horvath A, Pall N, Molnar K, et al.A nationwide study of the epidemiology of relapsing polychondritis.Clin Epidemiol.2016;8:211–230. doi:10.2147/CLEP.S91439Additional ReadingGulati A, Bagga A.Large vessel vasculitis.Pediatr Nephrol. 2010 Jun; 25(6):1037–1048. doi:10.1007/s00467-009-1312-9Lucerna A, Espinosa J.Acute atraumatic pinna (auricular) perichondritis.World J Emerg Med. 2018;9(2):152–153. doi:10.5847/wjem.j.1920-8642.2018.02.013
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Genetic and Rare Diseases Information Center (GARD).Relapsing polychondritis.Borgia F, Giuffrida R, Guarneri F, et al.Relapsing polychondritis: an updated review.Biomedicines. 2018 Sep;6(3):84. doi:10.3390/biomedicines6030084Sosada B, Loza K, Bialo-Wojcicka E.Relapsing polychondritis.Case Rep Dermatol Med.2014; 2014:791951. doi:10.1155/2014/791951Lepka FK, Chevalier X.Refractory relapsing polychondritis: challenges and solutions.Open Access Rheumatol.2018;10:1–11. doi:10.2147/OARRR.S142892Horvath A, Pall N, Molnar K, et al.A nationwide study of the epidemiology of relapsing polychondritis.Clin Epidemiol.2016;8:211–230. doi:10.2147/CLEP.S91439
Genetic and Rare Diseases Information Center (GARD).Relapsing polychondritis.
Borgia F, Giuffrida R, Guarneri F, et al.Relapsing polychondritis: an updated review.Biomedicines. 2018 Sep;6(3):84. doi:10.3390/biomedicines6030084
Sosada B, Loza K, Bialo-Wojcicka E.Relapsing polychondritis.Case Rep Dermatol Med.2014; 2014:791951. doi:10.1155/2014/791951
Lepka FK, Chevalier X.Refractory relapsing polychondritis: challenges and solutions.Open Access Rheumatol.2018;10:1–11. doi:10.2147/OARRR.S142892
Horvath A, Pall N, Molnar K, et al.A nationwide study of the epidemiology of relapsing polychondritis.Clin Epidemiol.2016;8:211–230. doi:10.2147/CLEP.S91439
Gulati A, Bagga A.Large vessel vasculitis.Pediatr Nephrol. 2010 Jun; 25(6):1037–1048. doi:10.1007/s00467-009-1312-9Lucerna A, Espinosa J.Acute atraumatic pinna (auricular) perichondritis.World J Emerg Med. 2018;9(2):152–153. doi:10.5847/wjem.j.1920-8642.2018.02.013
Gulati A, Bagga A.Large vessel vasculitis.Pediatr Nephrol. 2010 Jun; 25(6):1037–1048. doi:10.1007/s00467-009-1312-9
Lucerna A, Espinosa J.Acute atraumatic pinna (auricular) perichondritis.World J Emerg Med. 2018;9(2):152–153. doi:10.5847/wjem.j.1920-8642.2018.02.013
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