Polychondritis
| Classification according to ICD-10 | |
|---|---|
| M94 | Other cartilage diseases |
| M94.1 | Panchondritis (Recurrent Polychondritis) |
| ICD-10 online (WHO version 2019) | |
The relapsing polychondritis , and relapsing polychondritis , Panchondritis , systematized chondromalacia or polychondritis atropicans called, is a very rare cartilage disease rheumatic and immunological nature. Characteristic for polychondritis are chronically recurring ( recurrent ) cartilage inflammation, through which the cartilage tissue loses its strength, which can lead to deformation or failure of the organ function. The clinical picture was described in 1923 by the Austrian internist Rudolf von Jaksch and is also known as Meyenburg-Altherr-Uehlinger syndrome .
causes
The exact cause is unknown, but the syndrome is now classified as an autoimmune disease .
Symptoms
The disease can affect any organ in the body that contains cartilage. However, the focus is on joint involvement with arthritis and the involvement of the nasal and ear cartilage with recurrent non-bacterial perichondritis , often long-term with the development of a saddle nose or a cauliflower ear . Involvement of the larynx and tracheal cartilage can lead to breathing problems. In some patients, inflammatory eye diseases ( conjunctivitis , scleritis ), increasing hearing loss ( inner ear hearing loss , conductive hearing loss ), vascular diseases ( vasculitis ) and heart valve defects are also present or as initial symptoms . Non-specific symptoms can include fatigue, night sweats, and weight loss.
Diagnosis
- Rheumatological examination with determination of the inflammation parameters in the blood, rheumatoid factors , antinuclear antibodies , IgG autoantibodies against collagen type II. Laboratory parameters specific for the disease are not known, however.
- Ophthalmological examination
- ENT examination with hearing test, in the case of acute perichondritis, fine-tissue cartilage examination
- Cardiological examination with EKG (cardiac arrhythmia) and ultrasound examination (heart valve defect)
therapy
Due to the rarity of the disease, there is insufficient therapy experience. Most of the currently used preparations have only been tested in individual cases. In the acute phase of the inflammatory flare-up, cortisone is used to stop the inflammation and prevent life-threatening complications. Antirheumatic and immunomodulatory drugs replace cortisone as the disease progresses. In addition, the therapy depends on the organ involvement: If the airways swell when the laryngeal cartilage is diseased, a tracheotomy may be necessary. Heart valve disease may require valve replacement.
literature
- Bernhard Hellmich et al: Involvement in the airways in recurrent polychondritis. In: Journal of Rheumatology. 2003 Feb; 62 (1), pp. 73-79.
- Otto Braun-Falco among others: Dermatology and Venereology. 5th edition. Springer-Verlag, 2005, ISBN 3-540-40525-9 .
- Achim Franzen and others: Recurrent polychondritis. In: forum-ENT. Volume 2/2008, Omnimed publishing company
