Abstract
Relapsing polychondritis is an immune-mediated systemic disease characterized by recurrent episodes of inflammation of cartilaginous and proteoglycan-rich tissues, resulting in progressive anatomical deformation and functional impairment of the involved structures. Auricular and nasal chondritis and/or polyarthritis represent the most common clinical features, but potentially all types of cartilage may be involved. Because of the pleomorphic nature of the disease, with non-specific symptoms at the onset, the diagnosis of relapsing polychondritis is often delayed. In this review article we provide a comprehensive look into clinical presentation, laboratory and instrumental investigations, diagnostic criteria, and therapeutic options.
Highlights
Relapsing polychondritis (RP) is an immune-mediated systemic disease characterized by recurrent inflammatory episodes of cartilaginous and proteoglycan-rich tissues, including the elastic cartilage of the ear and nose, the hyaline cartilage of peripheral joints, the fibrocartilage at axial sites and the cartilage of the tracheobronchial tree, which result in progressive anatomical deformation and functional impairment of the involved structures
In this review article we provide a comprehensive look into clinical presentation, laboratory and instrumental investigations, diagnostic criteria, and therapeutic options, with a focus on the role of biologics in the management of refractory patients
Chondritis and polyarthritis are the most common clinical features of RP but, since inflammation of cartilaginous tissue potentially may occur at many anatomical districts, the disease often presents with various combinations of heterogeneous, only apparently unrelated, signs and symptoms
Summary
Relapsing polychondritis (RP) is an immune-mediated systemic disease characterized by recurrent inflammatory episodes of cartilaginous and proteoglycan-rich tissues, including the elastic cartilage of the ear and nose, the hyaline cartilage of peripheral joints, the fibrocartilage at axial sites and the cartilage of the tracheobronchial tree, which result in progressive anatomical deformation and functional impairment of the involved structures. In over 80% of patients, RP is disclosed by auricular chondritis and polyarthritis, though many organs can be potentially involved. Its onset is often insidious, with acute painful inflammatory crisis followed by spontaneous remission of variable duration. This may render diagnosis very difficult at an early stage, with therapeutic delay and consequent increased risk of permanent or life-threatening sequelae. In this review article we provide a comprehensive look into clinical presentation, laboratory and instrumental investigations, diagnostic criteria, and therapeutic options, with a focus on the role of biologics in the management of refractory patients
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