Introduction: The diagnosis of relapsing polychondritis is difficult as various manifestations may be encountered aside the characteristic episodes of recurrent chondritis.
Case Reports: From the retrospective analysis of the medical charts of patients presenting with relapsing polychondritis seen at Nîmes hospital between 1995 to 2006, four were selected for their original extra-cartilaginous manifestations. Case 1: relapsing polychondritis was diagnosed at the time of a thromboembolic event associated with a right uveitis, left temporomandibular arthritis and bilateral sensorineural deafness. Case 2: relapsing polychondritis occurred in a patient with history of bilateral sensorineural deafness with punctuated keratitis followed by a relapsing cutaneous leucocytoclastic vasculitis. Case 3: relapsing polychondritis associated with recurrent thromboembolic disease and a Sweet's syndrome and case 4: relapsing polychondritis presenting with a febrile erythema.
Conclusion: The reported observations highlight the difficulty of the initial diagnosis of relapsing chondritis and the variety of the extra-chondritis manifestations that could be observed in this disease.
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http://dx.doi.org/10.1016/j.revmed.2008.03.370 | DOI Listing |
Int J Rheum Dis
January 2025
Department of Internal Medicine, Hedi Chaker University Hospital, Sfax, Tunisia.
Int J Med Sci
January 2025
Department of Rheumatism and Immunology, Tianjin First Central hospital, Tianjin, China.
Autoimmune inner ear disease (AIED) is a rare condition characterized by immune-mediated damage to the inner ear, leading to progressive sensorineural hearing loss (SNHL) and vestibular symptoms such as vertigo and tinnitus. This study investigates the pathogenesis and therapeutic strategies for AIED through the analysis of three cases with different underlying autoimmune disorders: rheumatoid arthritis, relapsing polychondritis, and IgG4-related disease. The etiology of AIED involves complex immunopathological mechanisms, including molecular mimicry and the "bystander effect," with specific autoantibodies, such as those against heat shock protein 70 (HSP70), playing a potential role in cochlear damage.
View Article and Find Full Text PDFInt J Dermatol
December 2024
Department of Dermatology, International University of Health and Welfare, Chiba, Japan.
Rev Med Interne
December 2024
Service de médecine interne, CHI Poissy-St Germain, 10, rue du Champs Gaillard, 78300 Poissy, France.
Introduction: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic), recently described, due to a somatic mutation of the UBA1 gene and often associated with hemopathy, is characterized by systemic symptoms close to those described in Still's disease or relapsing polychondritis. There are also patients with hemopathy, presenting inflammatory symptoms reminiscent of those of VEXAS syndrome but without mutation of the UBA1 gene.
Case/discussion: Two male patients consulted for general signs, dermatological symptoms, arthralgia, chondritis and venous thrombosis, like patients in the French cohort suffering from VEXAS syndrome.
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