Relapsing polychondritis

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Relapsing polychondritis
Classification and external resources
ICD-10 M94.1
ICD-9 733.99
DiseasesDB 10248
eMedicine med/2000 derm/375
MeSH D011081

Relapsing polychondritis (also known as "Atrophic polychondritis,"[1] and "Systemic chondromalacia"[1]) is a multi-systemic condition characterized by inflammation and deterioration of cartilage. The often painful disease can cause deformity and be life-threatening when respiratory tract, heart valves or blood vessels are affected. The disease is not well known, is not well understood, and is often undiagnosed.

It is also known as chronic atrophic polychondritis, Meyenburg-Altherr-Uehlinger syndrome, von Meyenburg's disease, generalized chondromalacia, and systemic chondromalacia.

Presentation[edit]

Relapsing polychondritis may first present itself in any age group at any time. Some literature reports a slightly higher occurrence in females than males, while other authors assert that sex is not a statistically significant factor in the incidence of the disease. Manifestations of the disease often occur sporadically in episodes contributing to the difficulty of diagnosis. Relapsing polychondritis is one of many subclasses of disease in the area of rheumatology.

Though any cartilage in the body may be affected, in many cases the disease affects several areas while sparing others completely. Parts of the body where cartilage is found, and therefore potentially affected by polychondritis, include the ears, nose, throat, heart valves, joints and rib cage, sinus cavities among others. Several conditions may result from relapsing polychondritis, including type 3 tracheomalacia and vasculitis. One sign to look for is a painful, red and swollen ear, often sparing the ear lobe, and often described by patients as "hot red ears"; a photograph of which is shown here [2]

Symptoms reported by patients[edit]

According to medscape "The affected systems and symptoms reported in patients with relapsing polychondritis before and after diagnosis include the following:

  • General- Intermittent fever, weight loss, and skin rash (see Physical)
  • Audiovestibular - Sudden ear pain (unilateral or bilateral), inability to sleep on affected side, floppy ear, suddenly diminished hearing, tinnitus (occasional or persistent), otitis media, ear drainage, vertigo (with or without nausea and vomiting), and unsteadiness.
  • Musculoskeletal - Polyarthritis or monoarthritis, myalgias, back pain, rib pain, sternal pain, calf pain or claudication, and migratory or generalized arthralgias
  • Respiratory - Dyspnea, wheezing, cough, exercise intolerance, hoarseness, and recurrent infection
  • Gastrointestinal - Dysphagia
  • Nasal - Feeling of fullness across nasal bridge, saddle-shaped nose, mild epistaxis, and painful, red, and swollen nose
  • Ocular - Decreased visual acuity, conjunctivitis, episcleritis, scleritis, history of ocular inflammation, diplopia, and eyelid swelling
  • Cardiovascular- Chest pain, abdominal pain, history of pericarditis, abnormal heart rate or rhythm, syncope, and history of subacute myocardial infarction (found on ECG)
  • Central nervous system - Headache, ataxia, confusion, cranial nerve palsy, confusion, psychiatric signs, focal weakness/sensation changes, dementia, and seizures[3]"

Causes[edit]

Reasons for disease onset are not known. Although it is understood that generally people are born with the genetic predisposition to develop this autoimmune disease, which can be triggered due to environmental factors, there have been no studies proving this. As with many autoimmune disease it is hypothesized that there may be a genetic factor contributing to the disease. There is reported cases where this disease has been passed on from a biological parent.

Relapsing polychondritis may be an auto-immune disease[4] in which the human's body's immune system begins to attack and destroy the cartilage tissues in the body.

Diagnosis[edit]

There is no specific test for relapsing polychondritis. McAdam criteria is often used to establish the diagnosis. Clinicians look for the following six signs: (1) red, swollen ear, sparing the ear lobe, during acute phase, deformed ear after acute inflammation; (2) red, swollen, and stiff joints; (3) inflammation and/or deformity of cartilage of the nose, often resulting in saddle deformity; (4) inflammation of the eye; (5) inflammation and/or deformity of the cartilage supporting the upper airway (trachea)often accompanied by hoarseness and possibly a wheezing sound emanating from the throat; (6) damage to the cartilage in the inner ear, resulting in hearing loss. The diagnosis is made if 3 or more of the above signs are present, together with confirmation from the

labs[edit]

Patients presenting with acute episodes often have high levels of inflammatory markers such as erythrocyte sedimentation rate or c-reactive protein, ESR or CRP. Patients often have cartilage-specific antibodies present during acute relapsing polychondritis episodes. Antinuclear antibody reflexive panel, rheumatoid factor, and antiphospholipid antibodies are tests that may assist in the evaluation and diagnosis of autoimmune connective-tissue diseases.

biopsy[edit]

Biopsy of the cartilage tissue (for example, ear) may show tissue inflammation and destruction, and may help with the diagnosis. The Biopsy of cartilage in patients with relapsing polychondritis may demonstrate chondrolysis, chondritis, and perichondritis.

imaging studies[edit]

Imaging studies including MRI, ct scans, and X-rays may revealinflamation and/or damaged cartilage facilitating diagnosis.

Treatment[edit]

It is important to note there have been no controlled trials published regarding the therapy of relapsing polychondritis. Current treatment aims to control patient symptoms and slow down/stop further damage of cartilage and body.


Treatment plans typically involve suppression of the immune system with medicines. The first line drug is systemic steroid. It works fast to control symptoms and reduced inflammation, but is associated with a number of potentially serious side effects. Doctors typically starts patients on calcium, vitamin D and bisphosphonates to prevent osteoporosis while he or she is on steroids. A steroid-sparing medication (e.g. azathioprine or cyclophosphamide) is often given with the steroid. It will take weeks before this medication to reach it full effect, at which time, steroid can be slowly tapered. The patient will need close follow-up with a rheumatologist or internist.

There is anecdotal evidence to suggest that a low fat, whole foods plant based diet may be helpful in treating the disease, however more research is needed.

prognosis[edit]

"Many individuals may have mild symptoms, which recur infrequently, while others may have persistent problems that become debilitating or life-threatening. [5]

See also[edit]

References[edit]

  1. ^ a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. 
  2. ^ Image of polychondritis of the ear
  3. ^ http://emedicine.medscape.com/article/331475-clinical
  4. ^ "Relapsing Polychondritis: Autoimmune Disorders of Connective Tissue". Merck Manual Home Health Handbook. 
  5. ^ http://www.polychondritis.org/what-is-rp/


"Polychondritis" Medscape. Web. 13 August 2014. <http://emedicine.medscape.com/article/331475-overview#a0104>

External links[edit]