Relapsing polychondritis (RP) is a rare, multisystem autoimmune disease characterized by inflammation, structural damage, and impaired function of cartilaginous tissues throughout the body. In the craniofacial region, this rare disease has been reported to affect structures of the ear and nose; however, reports of temporomandibular joint (TMJ) involvement are scarce. A second uncommon disorder of cartilage is synovial chondromatosis (SC), a progressive and proliferative disorder of the synovial membrane associated with the formation of variably sized cartilaginous and calcified loose bodies, often causing dysfunction of the joints and enlargement of the joint capsule. It commonly affects the larger joints; TMJ involvement is uncommon. We present the case of a 45-year-old woman with previously diagnosed RP in whom right TMJ pathology subsequently developed, undergoing arthroscopy and biopsy followed by arthroplasty, which was proved to be SC, likely due to her autoimmune disease. To our knowledge, this is the first case describing concomitant SC of the TMJ presumably from pre-existing RP.
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http://dx.doi.org/10.1016/j.joms.2016.04.035 | DOI Listing |
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.
Immunotherapy
December 2024
Department of Clinical Immunology and Allergy, Flinders Medical Centre, Bedford Park, South Australia.
Relapsing polychondritis is rare and affects non-synovial fibrocartilage. Currently, there is a paucity of treatment algorithms, especially for those with refractory disease. A middle-aged man presented with polychondritis affecting the nose, ears, joints, and larynx.
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