ABSTRACT
Relapsing polychondritis (RP) is a rare, chronic multisystem disorder of unknown etiology characterized by recurrent episodic inflammation of cartilaginous structures that results in tissue destruction that can be life-threatening (I). All types of cartilage, including the elastic cartilage of the ears and nose, the hyaline cartilage of the tracheobronchial tree and peripheral joints, and the fibrocartilage of the axial skeleton may be involved. Inflammatory injury may also affect the eye, heart, blood vessels, and inner ear. Constitutional symptoms are common. Vasculitis may affect small, medium, or large vessels. There is a paucity of data regarding the epidemiology, incidence, prevalence, and mortality of this rare disease. No specific serological tests for RP have been identified, and the diagnosis is based on the characteristic clinical manifestations. Patients with RP have an increased frequency of HLA-DR4 (2,3), and often have coexistent autoimmune diseases (4). Relapsing polychondritis pursues a fluctuating and often progressive course with bouts of inflammation that may lead to permanent destruction of involved tissues. In a minority of patients, the disease may be self-limited. Glucocorticoids (GCs) are the mainstay of therapy during acute attacks. Cytotoxic agents such as cyclophosphamide (CYC), azathioprine (AZA), and methotrexate (MTX) have been used as additional therapy although no controlled studies have been performed with these agents. Overall, 74% of patients are alive 5 years, and 55% 10 years after disease onset (5).
