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Clinical manifestations of relapsing polychondritis

Clement J Michet, MD
Section Editor
Peter H Schur, MD
Deputy Editor
Paul L Romain, MD


Relapsing polychondritis (RPC) is an immune-mediated condition associated with inflammation in cartilaginous structures and other tissues throughout the body, particularly the ears, nose, eyes, joints, and respiratory tract. Approximately one-third of RPC cases occur in association with another disease, usually some form of systemic vasculitis, a connective tissue disorder (systemic autoimmune rheumatologic disease), or a myelodysplastic syndrome (MDS).

The clinical features and course of RPC vary considerably from patient to patient. Subtle, early manifestations often remain unrecognized for prolonged periods. As a result, the diagnosis is frequently obtained only after the emergence of classic features such as auricular inflammation, saddle-nose deformity, or other features of cartilage destruction. No known clinical or laboratory measures predict the expression of specific disease manifestations or the overall disease course.

The clinical features of RPC are reviewed here. The pathogenesis, pathology, diagnosis, and treatment of RPC are discussed separately. (See "Etiology and pathogenesis of relapsing polychondritis" and "Pathology of relapsing polychondritis" and "Diagnostic evaluation of relapsing polychondritis" and "Treatment of relapsing polychondritis".)


Relapsing polychondritis (RPC) is a rare disease that appears to occur among individuals of all races and age groups. Men and women are affected equally, but much about the epidemiology of RPC remains unknown because of the relatively small number of affected patients. RPC is most prevalent in Caucasians. The annual incidence has been estimated at 0.71, 2.0, and 3.5 per million persons from population studies in the United Kingdom, Hungary, and Minnesota, respectively [1-3]. Onset is most likely between the ages of 40 and 60, although RPC can occur in childhood (figure 1) [4].

RPC is not a familial disease, although studies indicate that some genetic contribution to susceptibility is likely. One case report suggested the possibility of placental transmission [5]. However, no other cases of this occurrence are confirmed, and the reported association may be secondary to chance or a confluence of other circumstances. In general, cases suggesting placental transmission are exceptionally rare, even if the mother may be experiencing active multisystem disease [5].

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Literature review current through: Sep 2017. | This topic last updated: Jan 09, 2017.
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