We present a 73-year-old Chinese male with bilateral relapsing, remitting orbital inflammatory disease associated with relapsing polychondritis. He first presented with right orbital inflammation that did not improve despite antibiotic treatment. Computer tomography (CT) of the orbits showed a soft tissue mass along the roof of the orbit, which was biopsied, revealing acute on chronic inflammation. There was complete resolution of his orbital inflammation within 2 weeks of initiating systemic steroid treatment. He subsequently developed recurrent bouts of left orbital inflammation. One year later, he was diagnosed with relapsing polychondritis and subsequently developed multiple myeloma seven years later. Comanagement with a rheumatologist will be helpful to achieve control of the disease with judicious use of immunosuppression. Long-term follow-up of the patient will be necessary to monitor for malignant transformation of the orbital lesion, as well as the development of other hematologic malignancies.
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http://dx.doi.org/10.3109/01676830.2014.902479 | 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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