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:[7][1][2] 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.[2] 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.[7] 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.[7] Last updated: 4/21/2015 This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Is Relapsing Polychondritis hereditary?

Is Relapsing Polychondritis hereditary?

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RELAPSING POLYCHONDRITIS

Synonyms:
Synonyms of Relapsing Polychondritis
chronic atrophic polychondritis.
generalized or systemic chondromalocia.
Meyenburg-Altherr-Uehlinger syndrome.
relapsing perichondritis.
von Meyenburg disease.

Types:
Not isolated yet

SYSTEMS AFECTED

Circulatory system
Circulatory system
Digestive system
Digestive system
Endocrine system
Endocrine system
Immune system
Immune system
Integumentary system
Integumentary system
Lymphatic system
Lymphatic system
Muscular system
Muscular system
Nervous system
Nervous system
Reproductive system
Reproductive system
Respiratory system
Respiratory system
Skeletal system
Skeletal system
Urinary system
Urinary system

Symptoms

  • There are no tests available that are specific for relapsing polychondritis (RP). A diagnosis is, therefore, generally based on the presence of characteristic signs and symptoms. For example, people may be diagnosed as having RP if they have three or more of the following features:[9]
  • Inflammation of the cartilage of both ears
  • Seronegative (negative for rheumatoid factor) polyarthritis (arthritis that involves 5 or more joints simultaneously)
  • Inflammation of the cartilage of the nose
  • Eye inflammation (conjunctivitis, episcleritis, scleritis, and/or uveitis)
  • Inflammation of the cartilage of the airway
  • Vestibular dysfunction (i.e. vertigo, hearing loss, tinnitus)
  • In some cases, a biopsy of affected tissue may be necessary to support the diagnosis.[9]

Treatments

  • The primary goals of treatment for people with relapsing polychondritis (RP) are to relieve present symptoms and to preserve the structure of the affected cartilage. The main treatment for RP is corticosteroid therapy with prednisone to decrease the severity, frequency and duration of relapses. Higher doses are generally given during flares, while lower doses can typically be prescribed during periods of remission. Other medications reported to control symptoms include dapsone, colchicine, azathioprine, methotrexate, cyclophosphamide, hydroxychloroquine, cyclosporine and infliximab.[5][10]
  • People who develop severe heart or respiratory complications may require surgery.[6]
  • Specialist who treat it

    Rheumatology
    Natural treatment of Relapsing Polychondritis

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    ICD9 and ICD10 codes of Relapsing Polychondritis

    ICD10 code of Relapsing Polychondritis and ICD9 code

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    Living with Relapsing Polychondritis

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    Stories of Relapsing Polychondritis

    STORY
    Relapsing Polychondritis stories
    The first time I noticed symptoms, I thought I was having an allergic reaction to something at work. My ears became so red and inflamed and hurt so bad. I let it go on for almost 3 days before finally going to a hospital where I was diagnosed with re...
    Relapsing Polychondritis stories
    It began with dry nose, nose bleeding and what my GP thought was a sinus infection. Antibiotics and prednisone perscribed, helped for a while, then reoccurred. Ear then swelled over twice the size - red, shiny and moist, GP again said outer ear infec...
    Relapsing Polychondritis stories
    i had progressively worsening breathing issues for many years before my diagnosis of rp.  I went through many specialists and tests and no one could identify why I was having breathing issues.  I had no outward signs of rp until one day my ...
    Relapsing Polychondritis stories
    I have had strange symptoms before this came to light, but during my last trimester of my pregnancy with my youngest son, I had a severe respiratory infection that never seemed to get better. I have had exercise induced asthma since I was young and t...
    Relapsing Polychondritis stories
    I was officially diagnosed with RP 2 yrs ago. No one else in my family has it. It started approximately 5 years ago with ear flares, jumping from one to the other. My PCP happened to go to a conference where RP was discussed, and soon contacted me to...

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    Relapsing Polychondritis forum

    QUESTION
    Question 666
    I don't think RP is as rare as we are led to believe. It seems many symptoms are unrelated. You go to an ortho doc for knee/joint pain. You go to an ENT for ears and throat. You go to an ophthalmologist for eyes. You go to a dermatologist for skin. T...

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    Statistics of Relapsing Polychondritis

    42 people with Relapsing Polychondritis have taken the SF36 survey. Mean of Relapsing Polychondritis is 1395 points (39 %). Total score ranges from 0 to 3,600 being 0 the worst and 3,600 the best. Take the SF36 Survey

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