Relapsing polychondritis: A clinical review*

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Abstract

Objective: This study comprehensively reviews the literature related to relapsing polychondritis (RP). Methods: A detailed search via MEDLINE (PubMed) was performed using relapsing polychondritis as the key term. Relevant articles were analyzed with a focus on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP. Results: RP is a rare episodic and progressive inflammatory disease of presumed autoimmune etiology first described in 1923. RP affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. The diagnosis of RP is based on the presence of clinical criteria. A standardized therapeutic protocol for RP has not been established. Nonsteroidal anti-inflammatory drugs, dapsone and/or colchicine, may control disease activity in some patients. In other patients, immunosuppressive drugs and prednisone have been effective. RP is a potentially lethal disease; pulmonary infection, systemic vasculitis, airway collapse, and renal failure are the most common causes of death. Earlier studies indicate survival rates between 70% at 4 years and 55% at 10 years. In a recent study, a survival rate of 94% at 8 years may be due to improved medical and surgical management. Conclusions: RP is a rare, multisystemic, and potentially fatal disease. The pathogenesis and optimal therapeutic approach to patients with RP is poorly understood. Semin Arthritis Rheum 31:384-395. Copyright 2002, Elsevier Science (USA). All rights reserved.

Section snippets

Methods

A detailed search of literature published between January 1966 and November 2001 via MEDLINE (PubMed) was performed using relapsing polychondritis as the key word. Relevant articles published in peer-reviewed journals were analyzed. These articles focused on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP. The ocular manifestations of RP were emphasized.

Epidemiology

Relapsing polychondritis is rare. Luthra (10) estimated the annual incidence as 3.5 cases per million in Rochester, NY. More than 600 cases have been reported worldwide. The average age at the time of diagnosis is 44 to 51 years (11), but may appear as early as 5 and as late as 84 years of age (12). The male-to-female ratio has been estimated to be between 1:1 and 1:3 (11).

A single congenital case, in which a pregnant woman with RP gave birth to a child with tender red knees and a saddle nose,

Etiology and pathogenesis

The etiology of RP is unknown, but the pathogenesis seems to be an immunologic reaction to type II collagen. Type II collagen is present in the sclera of the eye and in cartilage. Patients with RP have demonstrated both autoantibodies and cellular immune reactions to type II collagen 15, 16, 17, 18.

Cellular immune reaction has been described against cartilage extracts by means of lymphocyte transformation or macrophage migration inhibition methods (18). Foidart et al (17) and Giroux et al (19)

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    *

    Address reprint requests to C. Stephen Foster, MD, FACS, Uveitis and Immunology Service, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114. E-mail: [email protected].

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