2013
DOI: 10.1016/j.rdc.2013.03.002
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Relapsing Polychondritis

Abstract: Relapsing polychondritis (RP) is a rare systemic autoimmune disease characterized by episodic, progressive inflammatory destruction of cartilage. It can occur as an overlap syndrome in patients with other rheumatologic conditions. The disease usually follows an indolent relapsing-remitting course, but occasionally it can progress rapidly and even cause death. Although auricular or nasal chondritis or peripheral arthritis without other significant organ involvement are usually treated with low-dose corticostero… Show more

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Cited by 52 publications
(53 citation statements)
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“…22 Relapsing polychondritis is a rare autoimmune disorder characterized by episodic, progressive inflammatory destruction of cartilage. 27 The exact etiology and physiopathology of the disease has not been established. 28 It affects cartilaginous structures including ears, nose, tracheobronchial tree, and joints; it can also involve noncartilaginous tissues rich in proteoglycans such as the eyes, inner ear, heart, blood vessels, and kidney.…”
mentioning
confidence: 99%
See 1 more Smart Citation
“…22 Relapsing polychondritis is a rare autoimmune disorder characterized by episodic, progressive inflammatory destruction of cartilage. 27 The exact etiology and physiopathology of the disease has not been established. 28 It affects cartilaginous structures including ears, nose, tracheobronchial tree, and joints; it can also involve noncartilaginous tissues rich in proteoglycans such as the eyes, inner ear, heart, blood vessels, and kidney.…”
mentioning
confidence: 99%
“…28 It affects cartilaginous structures including ears, nose, tracheobronchial tree, and joints; it can also involve noncartilaginous tissues rich in proteoglycans such as the eyes, inner ear, heart, blood vessels, and kidney. 27,28 Diagnostic criteria were established by Michet et al 29 which require the presence of proven inflammation in at least two of three of the auricular, nasal, or laryngotracheal cartilages, or proven inflammation in one of these cartilages plus two other signs, including ocular inflammation, vestibular dysfunction, seronegative inflammatory arthritis, or hearing loss. 28,29 Our case is consistent with the diagnosis of TINU syndrome and was confirmed histopathologically with renal biopsy associated with typical bilateral uveitis.…”
mentioning
confidence: 99%
“…Clinical features include unilateral or bilateral painful erythematous swelling of the affected cartilage; the earlobe is typically not affected [ 2 ] . Apart from bilateral auricular chondritis, the condition may be associated with ophthalmitis and audiovestibular lesions [ 3 ] ; neither was present in our patient. As in the case of our patient, histology of relapsing polychondritis is characterized by a mixed perichondral infi ltrate predominantly made up of lymphocytes and plasma cells [ 1,3 ] .…”
Section: Discussionmentioning
confidence: 44%
“…Aseptic meningitis, with thickening of the meninges, lymphocytic meningoencephalitis, rhomboencephalitis and cerebral aneurysms can also occur. 1,[10][11][12] In our case we made the diagnosis solely on clinical grounds, as there was evidence on physical examination of chondritis of both ear lobes, sero-negative polychondritis and neurological compromise with aseptic meningitis. About 25% of patients die in up to five years following diagnosis; laryngotracheal involvement and cardiovascular complications are the leading causes …”
Section: Discussionmentioning
confidence: 97%