AI Article Synopsis
- The case discusses a unique occurrence of relapsing polychondritis (RP) presenting as seronegative limbic encephalitis in a 70-year-old woman with a relevant medical history.
- The diagnosis was achieved through collaboration among various medical specialists, highlighting the complexity of the condition.
- Timely treatment with steroids and azathioprine resulted in significant improvements in the patient's physical and cognitive functions, underscoring the need for a coordinated approach in managing such disorders.
Article Abstract
The presented case highlights a rare instance of relapsing polychondritis (RP) manifesting as seronegative limbic encephalitis, an uncommon neurological complication. A 70-year-old female patient with a history of RP-related inflammation, along with neuropsychiatric symptoms, was diagnosed through multidisciplinary collaboration. Swift administration of steroid therapy, followed by azathioprine, led to remarkable physical and cognitive recovery. This case emphasises the importance of a multidisciplinary approach in diagnosing and treating complex autoimmune disorders with neurological manifestations.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11046591 | PMC |
| http://dx.doi.org/10.7861/clinmed.2023-0430 | DOI Listing |
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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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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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