Περίληψη:
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.
Συγγραφείς:
Letko, E
Zafirakis, P
Baltatzis, S
Voudouri, A and
Livir-Rallatos, C
Foster, CS
