Relapsing polychondritis is a rare autoimmune disease characterized by inflammation of cartilaginous tissues. It may be associated with systemic and cerebral vasculitis and exceptionally with ischemic stroke. Brain infarction associated with internal carotid artery thrombus, in a setting of relapsing polychondritis, has never been reported. We present a 52-year-old man without any known risk factors for stroke, treated with prednisone and azathioprine for relapsing polychondritis, who presented a minor left hemisphere stroke. Ultrasound of the neck vessels revealed an isoechogenic thrombus in the left internal carotid artery superimposed on a smooth moderately stenosing isoechogenic atheroma of the carotid bulb. The patient was treated with high-dose tinzaparin and was followed with serial ultrasound. After 16 days, the thrombus demonstrated a hypoechogenic core surrounded by a hyperechogenic rim and the following day it resolved completely. Thrombus formation on a small unruptured plaque may reflect involvement by relapsing polychondritis of the intimal proteoglycans that hold a role in the development of atheromatosis.
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View Article and Find Full Text PDFArticle Synopsis
- Relapsing polychondritis (RP) is a rare autoimmune disorder that causes recurring inflammation in cartilaginous tissues, affecting areas like the ears, nose, and joints.
- A 68-year-old woman without prior health issues showed symptoms like hoarseness, polyarthritis, and nasal/ear involvement, leading to her RP diagnosis using the Modified McAdam criteria.
- She was treated with intravenous cyclophosphamide and oral prednisolone, which improved her condition, while osteoporosis was treated with zoledronic acid, highlighting RP's role in patient assessments involving similar symptoms.
VEXAS syndrome initially presenting as relapsing polychondritis and progressing into neutrophilic dermatosis with systemic symptoms over a 5-year period.
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[VEXAS-like auto inflammatory syndrome: 2 cases].
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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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