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Genetic and Rare Diseases Information Center (GARD)

Relapsing polychondritis

Other Names for this Disease
  • Chronic atrophic polychondritis
  • Recurrent polychondritis
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What are the signs and symptoms of Relapsing polychondritis?

Relapsing polychondritis (RP) is characterized by recurrent inflammation of cartilage (the tough but flexible tissue that covers the ends of bones at a joint) and other tissues throughout the body. The features of the condition and the severity of symptoms vary significantly from person to person, but may include:[1][2][3]
  • Ear: The ears are the most commonly affected body part. Symptoms include a sudden onset of pain, swelling, and tenderness of the cartilage of one or both ears. The pinna usually loses firmness and becomes floppy; hearing impairment may also occur. Inflammation of the inner ear may also cause nausea, vomiting, dizziness, and/or ataxia.
  • Joint: The second most common finding is joint pain with or without arthritis.
  • Eye: Affected people may experience episcleritis, uveitis and/or scleritis. Scleritis may lead to a bluish or dark discoloration of the sclera (white of the eye) and may even be associated with vision loss in severe cases. Proptosis (bulging out of one or both eye balls) may also be a symptom of RP.
  • Nose: Nasal cartilage inflammation may lead to stuffiness, crusting, rhinorrhea, epistaxis (nose bleeds), compromised sense of smell and/or saddle nose deformity (a condition where the nose is weakened and thus "saddled" in the middle).
  • Airways: Inflammation may affect the larynx, trachea (windpipe), and bronchi (tubes that branch off the trachea and carry air to the lungs). Airway involvement may lead to a cough, wheezing, hoarseness and recurrent infections. It can become life-threatening if not properly diagnosed and managed.

Less commonly, RP may affect the heart, kidneys, nervous system, gastrointestinal tract, and/or vascular (veins) system. Nonspecific symptoms such as fever, weight loss, malaise, and fatigue may also be present.[3]

In approximately one third of affected people, RP is associated with other medical problems. Conditions reportedly associated with RP include hematological disease (including Hodgkin's lymphoma and myelodysplastic syndromes); gastrointestinal disorders (including Crohn's disease and ulcerative colitis); endocrine diseases (including diabetes mellitus type 1 and thyroid disorders) and others.[1]

Episodes of RP may last a few days or weeks and typically resolve with or without treatment. However, it is generally progressive, and many people have persistent symptoms in between flares.[1]
Last updated: 4/21/2015

The Human Phenotype Ontology provides the following list of signs and symptoms for Relapsing polychondritis. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Abnormality of the nose 90%
Arthralgia 90%
Arthritis 90%
Chondritis 90%
Chondritis of pinna 90%
External ear malformation 90%
Abnormality of temperature regulation 50%
Abnormality of the aortic valve 50%
Abnormality of the pericardium 50%
Abnormality of the voice 50%
Aneurysm 50%
Autoimmunity 50%
Cartilage destruction 50%
Cataract 50%
Dilatation of the ascending aorta 50%
Inflammatory abnormality of the eye 50%
Limitation of joint mobility 50%
Osteolysis 50%
Periorbital edema 50%
Proptosis 50%
Sinusitis 50%
Vasculitis 50%
Vertigo 50%
Abnormality of the endocardium 7.5%
Abnormality of the liver 7.5%
Abnormality of the mitral valve 7.5%
Abnormality of the myocardium 7.5%
Abnormality of the oral cavity 7.5%
Anemia 7.5%
Arrhythmia 7.5%
Arterial thrombosis 7.5%
Conductive hearing impairment 7.5%
Congestive heart failure 7.5%
Coronary artery disease 7.5%
Cranial nerve paralysis 7.5%
Encephalitis 7.5%
Gangrene 7.5%
Glomerulopathy 7.5%
Hematuria 7.5%
Hemiplegia/hemiparesis 7.5%
Hypermelanotic macule 7.5%
Incoordination 7.5%
Laryngomalacia 7.5%
Myelodysplasia 7.5%
Proteinuria 7.5%
Recurrent respiratory infections 7.5%
Renal insufficiency 7.5%
Respiratory insufficiency 7.5%
Sensorineural hearing impairment 7.5%
Skin ulcer 7.5%
Subcutaneous hemorrhage 7.5%
Thrombophlebitis 7.5%
Tinnitus 7.5%
Tracheal stenosis 7.5%
Tracheomalacia 7.5%
Urticaria 7.5%

Last updated: 7/1/2016

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.

  1. Alexandros A. Drosos. Relapsing polychondritis. Orphanet Encyclopedia. October 2004; Accessed 12/19/2011.
  2. Puéchal X, Terrier B, Mouthon L, Costedoat-Chalumeau N, Guillevin L, Le Jeunne C. Relapsing polychondritis. Joint Bone Spine. March 2014; 81(2):118-124.
  3. Clement J Michet, MD. Clinical manifestations of relapsing polychondritis. UpToDate. February 2015; Accessed 4/21/2015.
  4. Nicholas Compton, MD. Polychondritis. Medscape Reference. March 2015;

Other Names for this Disease
  • Chronic atrophic polychondritis
  • Recurrent polychondritis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.