Relapsing polychondritis: Difference between revisions
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The earliest abnormality of hyaline and elastic cartilage noted histologically is basophilic staining indicating depletion of proteoglycan from the cartilage matrix. Inflammatory infiltrates are found adjacent to involved cartilage and consist predominantly of mononuclear cells and occasional plasma. | |||
Immunologic mechanisms play an important role in the pathogenesis of relapsing polychondritis.Humoral and cell mediated immunity play an important role in the pathogenesis.Immunoglobin and complement deposits are found at sites of inflammation. | |||
Revision as of 18:21, 18 November 2012
| Relapsing polychondritis | |
| ICD-10 | M94.1 |
|---|---|
| ICD-9 | 733.99 |
| DiseasesDB | 10248 |
| eMedicine | med/2000 derm/375 |
| MeSH | D011081 |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ,Assistant Editor-in-Chief: Soumya Sachdeva
Overview
Relapsing polychondritis is a condition where cartilage deteriorates.
It is also known as Chronic atrophic polychondritis, Meyenburg-Altherr-Uehlinger syndrome, von Meyenburg's disease, Generalized chondromalacia, or Systemic chondromalacia.
Causes
Reasons for disease onset are not known.
It is considered to possibly be an auto-immune disease[1] in which the human's body's immune system begins to attack and destroy the cartilage tissues in the body.
Pathophysiology
The earliest abnormality of hyaline and elastic cartilage noted histologically is basophilic staining indicating depletion of proteoglycan from the cartilage matrix. Inflammatory infiltrates are found adjacent to involved cartilage and consist predominantly of mononuclear cells and occasional plasma. Immunologic mechanisms play an important role in the pathogenesis of relapsing polychondritis.Humoral and cell mediated immunity play an important role in the pathogenesis.Immunoglobin and complement deposits are found at sites of inflammation.
Diagnosis
Onset
While the disease can come on at various times, most frequent time for onset is in the late 40's to early 50's. Some literature reports a slightly higher occurrence in females than males, while other literature asserts that sex is apparently not a statistically significant factor in the occurrence rate of the disease. Polychondritis is one of many subclasses of disease in the area of Rheumatology.
Presentation
All cartilage areas can be affected, though in many cases the disease will affect several areas where cartilage is found in the body, and leave others entirely alone. Parts of the body with cartilage, and therefore potentially affected by polychondritis, include the ears, nose, throat, heart valves and of course all areas where musculo-skeletal tissues are connected by cartilage. Specific resultant conditions may include Type 3 Tracheomalacia and Vasculitis.
Symptoms
- Decreased joint mobility
- Joint erythema
- Joint inflammation
- Joint swelling
- Pain on movement of joint
Differential Diagnosis
- Cauliflower ear
- Chondritis
- Chondromdermatitis
- Hansen's disease
- Nodularis helicis
- Perichondritis
- Rhinophyma
- Skin cancer
- Syphilis
Treatment
Treatment plans typically involve suppression of the immune system with medicines, which often result in a side effect of increasing the risk of other infections.
References
External links
- Polychondritis Educational Society, Ltd. (PES)
- Dr. D. E. Trentham research paper
- MedicineNet.com
- The Polychondritis Group - Support Group
Template:Osteochondropathy
Template:Diseases of the musculoskeletal system and connective tissue
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