Relapsing polychondritis is a relatively uncommon disease characterized by recurrence of progressive inflammation of cartilaginous structures. Laryngotracheal involvement occurs in 50-70% of cases, and occasionally results in acute airway obstruction. We reported a case of relapsing polychondritis with severe tracheal stenosis that was treated by the placement of two expandable metallic stents. A 58-year-old man was hospitalized with fever and breathing difficulties. A tracheostomy was performed and a silicone T-tube was placed. A diagnosis of relapsing polychondritis was made based on the biopsy from auricular and tracheal cartilages. Two expandable metallic stents were placed in the trachea to bilateral main bronchus. This dilated the airway lumen and resulted in the dyspnea disappearing completely. However, 18 months later a sudden massive hemorrhage occurred through the tracheostoma, and he died of respiratory failure. Autopsy showed a tracheoinnominate artery fistula. We discuss the management of relapsing polychondritis with severe tracheal stenosis.
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http://dx.doi.org/10.1016/s0385-8146(02)00133-5 | 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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