Relapsing polychondritis (RP) is a rare, progressive immune-mediated systemic inflammatory disease of unknown etiology, characterized by recurrent inflammation of cartilaginous structures. Approximately 30% of RP cases are associated with other autoimmune diseases. However, the co-occurrence of RP and Crohn disease (CD) has rarely been reported. Herein, we present a 35-year-old woman diagnosed with RP and CD, who was refractory to initial conventional medications, including azathioprine and glucocorticoid, but who subsequently responded to infliximab (IFX). For both diseases, remission was sustained with IFX. There has been no previous report regarding the successful treatment of co-existing RP and CD with IFX.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7787902 | PMC |
| http://dx.doi.org/10.12701/yujm.2020.00304 | 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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December 2024
Department of Dermatology, International University of Health and Welfare, Chiba, Japan.
[VEXAS-like auto inflammatory syndrome: 2 cases].
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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.
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