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 condition characterized by inflammation and deterioration of cartilage. Although the disease usually causes pain and deformity if unrecognized and untreated, it can be life-threatening when respiratory tract, heart valves or blood vessels are affected. The disease is not well known, and 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]

While the disease may first present at various times, it most frequently presents in the late 40's to early 50's. Children and young adults are also diagnosed with this disease, although less often. 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. Relapsing polychondritis is one of many subclasses of disease in the area of rheumatology. This disease can begin with a few mild symptoms, hot red ears and ear canal inflammation or joint pain and progress over months to years to become more serious with inflammation spreading to various other sites of cartilage causing more severe pain.

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]

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 disease, which can be triggered due to environmental factors. Consequently this disease can be genetic, there is reported cases where this disease has been passed on from a biological parent.

Relapsing polychondritis may be an auto-immune disease[3] 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. Patient presented with acute episode often has high level of inflammatory markers (erythrocyte sedimentation rate or c-reactive protein, ESR or CRP). Biopsy of the cartilage tissue (for example, ear) shows tissue inflammation and destruction, and may help with the diagnosis. 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 biopsy.

Treatment[edit]

Treatment plans typically involve suppression of the immune system with medicines. The first line drug is systemic steroid, prednisone orally or medroxyprednisolone intravenously, depending how sick the patient is. 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.

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. ^ "Relapsing Polychondritis: Autoimmune Disorders of Connective Tissue". Merck Manual Home Health Handbook. 

External links[edit]