Diagnosis and classification of relapsing polychondritis.
Relapsing polychondritis is a rare and potentially fatal autoimmune disease of unknown etiology, characterized by inflammation and destruction of different cartilaginous structures, including the ear, nose, larynx, trachea, bronchi, peripheral joints, eye, heart and skin, with high risk of misdiagnosis. The spectrum of clinical presentations is protean and may vary from intermittent episodes of painful and disfiguring auricular and nasal chondritis or polyarthritis to severe progressive multi-organ damage. A laryngotracheobronchial involvement appears in nearly half of patients and is complicated by local obstructions, which may be life-threatening. A highly medical specialized approach is required for diagnosis of relapsing polychondritis. This review comprehensively examines the literature related to the clinical sceneries of the disease and focuses on both diagnostic tools used in clinical studies and recent findings related to its etiopathogenesis.
Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy. Electronic address: cantariniluca@hotmail.com.
Luca
L
Cantarini
Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy.
Antonio
A
Vitale
Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy.
Maria Giuseppina
MG
Brizi
Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy; Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Padova, Padova, Italy.
Francesco
F
Caso
Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy.
Bruno
B
Frediani
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Padova, Padova, Italy.
Leonardo
L
Punzi
Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy.
Mauro
M
Galeazzi
Institute of Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy.
Donato
D
Rigante
D001327:Autoimmune Diseases / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D007249:Inflammation / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D011081:Polychondritis, Relapsing / Q000145:classification* / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology
Cartilage disorder; Relapsing polychondritis; Type II collagen