Relapsing polychondritis

The Relapsing polychondritis, relapsing polychondritis also, Panchondritis, Systematized chondromalacia or polychondritis atropicans, is a very rare cartilage disease rheumatic and immunological nature. Characteristic of the polychondritis are chronically recurrent (recurrent ) cartilage inflammation, through which the cartilage loses its strength, which can lead to deformation or failure of organ function. The disease was described in 1923 by the Austrian Rudolf von Jaksch internists and is also known as Meyenburg Altherr - Uehlinger syndrome.

Causes

The exact cause is unknown, the syndrome but is now associated with autoimmune diseases.

Symptoms

The disease can affect all organs of the body that contain cartilage. In the foreground, however, are joint investments with arthritis and the involvement of the nose and ear cartilage with recurrent non-bacterial perichondritis, often long-term training with a saddle nose or a cauliflower ear. The involvement of the larynx and trachea cartilage can cause breathing problems. Some patients are in addition or as an initial symptom inflammatory eye disease (conjunctivitis, scleritis ), increasing hearing loss ( sensorineural hearing loss, conductive hearing loss ), vascular diseases (vasculitis ) and valvular heart disease. Non-specific symptoms may be fatigue, night sweats and weight loss.

Diagnostics

• Rheumatological examination with determination of inflammatory markers in the blood, rheumatoid factor, antinuclear antibodies, IgG autoantibodies against collagen type II but are not known for the disease -specific laboratory parameters.
• Ophthalmologic examination
• ENT examination with hearing test, histological examination in acute perichondritis cartilage.
• Cardiac examination with ECG (heart rhythm disorder) and ultrasound ( valvular heart disease ).

Therapy

Due to the rarity of the disease, there are only limited therapeutic experience. All preparations currently used were mostly tested only in individual cases. In the acute phase of inflammation has cortisone is used to stop the inflammation and prevent life-threatening complications. Antirheumatic and immunomodulatory drugs replace the cortisone with increasing progression of the disease. For the rest, the treatment depends on the organ involvement: If disease of the larynx cartilage swell up the airways, a tracheotomy may be necessary. Diseases of the heart valves can make a valve replacement required.