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Erschienen in: Wiener Medizinische Wochenschrift 9-10/2017

31.03.2017 | main topic

Relapsing polychondritis: a chameleon among orphan diseases

verfasst von: Sabine Schumacher, MD, Herwig Pieringer, MD, MBA

Erschienen in: Wiener Medizinische Wochenschrift | Ausgabe 9-10/2017

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Summary

Relapsing polychondritis (RPC) is a rare disease with recurrent episodes of inflammation of cartilage tissue leading to fibrosis and organ damage. Despite unknown etiology, there is some evidence of a genetic predisposition. The clinical presentation is heterogeneous and an association with other autoimmune disorders such as rheumatoid arthritis or different forms of vasculitis has been described. All organ systems containing cartilage can be affected, such as ear, nose, joints, trachea, aorta, and coronary arteries. Given the broad spectrum of potential manifestations, a variety of medical specialists may be involved in the management of RPC patients. As establishing the diagnosis of RPC may be difficult, an interdisciplinary approach may be preferable. Treatment options include glucocorticoids, dapsone, disease-modifying antirheumatic drugs, and biologics. Prognosis is as heterogeneous as the clinical picture, depending on the severity of organ damage. In this paper we give an overview of the current knowledge with regard to pathogenesis, clinical picture, diagnosis, and therapy of RPC.
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Metadaten
Titel
Relapsing polychondritis: a chameleon among orphan diseases
verfasst von
Sabine Schumacher, MD
Herwig Pieringer, MD, MBA
Publikationsdatum
31.03.2017
Verlag
Springer Vienna
Erschienen in
Wiener Medizinische Wochenschrift / Ausgabe 9-10/2017
Print ISSN: 0043-5341
Elektronische ISSN: 1563-258X
DOI
https://doi.org/10.1007/s10354-017-0559-1

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