Relapsing polychondritis.

Beata Sosada,Katarzyna Loza,Ewelina Bialo-Wojcicka

doi:10.1155/2014/791951

Beata Sosada, Katarzyna Loza + Show 1 more

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https://doi.org/10.1155/2014/791951

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Abstract

Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. Additional clinical features include audiovestibular dysfunction, ocular inflammation, vasculitis, myocarditis, and nonerosive arthritis. Although the cause remains unknown, the etiology is suspected to be autoimmune. We describe a case of a 31-year-old woman with a four-month history of bilateral auricular and nasal chondritis. Infectious and neoplastic diseases were excluded by imaging and laboratory examinations. RP was diagnosed based on three McAdam's criteria. The patient was medicated with oral prednisolone and methotrexate with positive clinical response. In this case clinical history and detailed physical examination were fundamental in concluding the correct diagnosis and administrating the appropriate medication.

Highlights

Relapsing polychondritis (RP) is a rare inflammatory disease primarily affecting the cartilaginous structures of the ear, nose, joints, tracheobronchial tree, and cardiovascular system
RP is an autoimmune disease in which target antigens are still unknown
Studies [8, 9] have shown that 33% of patients with RP had circulating antibodies of type II collagen in the active phase of the disease and their titres corresponded to the disease activity

Summary

Introduction

Relapsing polychondritis (RP) is a rare inflammatory disease primarily affecting the cartilaginous structures of the ear, nose, joints, tracheobronchial tree, and cardiovascular system. The first case of RP was described in 1923 by Jaksch-Wartenhorst [1]. The McAdam’s criteria were the initial diagnostic criteria of RP [3] and required meeting three out of six of the following: bilateral auricular chondritis, nonerosive seronegative inflammatory arthritis, nasal chondritis, ocular inflammation, respiratory tract chondritis, and audiovestibular damage. The diagnosis of RP relies mostly on the criteria established by Michet et al [5] which require the presence of a proven inflammation in at least two of three of the auricular, nasal, or laryngotracheal cartilages or the proven inflammation in one of these cartilages plus two other signs, including ocular inflammation, vestibular dysfunction, seronegative inflammatory arthritis, or hearing loss (Table 1). A rare disease RP is described occurring extremely rarely in young women

Case Report

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