Relapsing Polychondritis

Peter D. Kent; Clement J. Michet, Jr; Harvinder S. Luthra

Disclosures

Curr Opin Rheumatol. 2004;16(1) 

In This Article

Abstract and Introduction

Abstract

Relapsing polychondritis is a unique, rare autoimmune disorder in which the cartilaginous tissues are the primary targets of destruction but the immune damage can spread to involve noncartilaginous tissues like the kidney, blood vessels, and so forth. The manifestations of the disease can take many different forms and the pathogenesis is still unclear. It may occur in a primary form or it may be associated with other disease states. This article summarizes important aspects of the disease with a focus on recent information regarding clinical manifestations, disease associations, pathogenesis, and advances in therapeutics.

Introduction

Relapsing polychondritis was first described 80 years ago, and although much has been written about this unusual disease, there continues to be much that we do not know. Although clinical observations have been well described, some of these reflect manifestations of associated diseases. The pathogenesis remains unknown, although immune abnormalities observed over the years led us to classify it as an autoimmune disease. Some of the difficulties involved in studying this disease relate to its rarity (making it difficult to study patients with a similar phenotype), the lack of appropriate animal models, and the lack of consistent laboratory abnormalities, all of which lead to difficulty in developing a clear working hypothesis. Ongoing efforts in this direction may soon produce results. The lack of substantial populations in any one center's therapeutic studies leave us with generic approaches to treatment, as is true of many immune-mediated inflammatory diseases. With the advances in our ability to investigate molecular mechanisms of diseases, and understanding the role of genomics and proteomics, we anticipate major advances in our understanding of this syndrome in the near future.

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