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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Relapsing polychondritis


Other Names for this Disease

  • Chronic atrophic polychondritis
  • Recurrent polychondritis
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Symptoms

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What are the signs and symptoms of relapsing polychondritis?

Relapsing polychondritis (RP) has been associated with a wide variety of conditions, signs and symptoms. Individuals with RP generally have a sudden onset of symptoms, typically involving the external ear and experiencing pain, swelling, redness and tenderness. Episodes may last a few days or weeks and typically resolve with or without treatment. 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. The second most common finding is joint pain with or without arthritis. About a third of affected individuals have inflammation of the blood vessels (vasculitis) and autoimmune rheumatic diseases such as rheumatoid arthritis and lupus. Ocular (eye) findings occur in about 60% and may include inflammation or irritation of various parts of the eye(s) and/or other findings. Respiratory symptoms and non-specific skin disorders (present in about 50% of affected individuals) are also common. Other findings that have been reported, but occur less commonly, include neurological abnormalities, cardiovascular (heart) manifestations and renal disease; renal disease typically indicates a worse prognosis. Other conditions reportedly associated with RP include hematological disease (including Hodgkin's lymphoma, myelodysplastic syndromes and others); gastrointestinal disorders (including Crohn's disease, ulcerative colitis and others); endocrine diseases (including diabetes mellitus type 1 and thyroid disorders) and others. RP is generally progressive, and many individuals have persistent symptoms in between flares. Most affected individuals develop some degree of disability over time.[1]
Last updated: 12/19/2011

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: 9/2/2014

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.


References
  1. Alexandros A. Drosos. Relapsing polychondritis. Orphanet Encyclopedia. October 2004; http://www.orpha.net/data/patho/GB/uk-RP.pdf. Accessed 12/19/2011.


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.