About a half of all patients with relapsing polychondritis show airway involvement, which is a major cause of morbidity and mortality from this disease. FDG-PET/CT is useful in the differential diagnosis of relapsing polychondritis from asthma.
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http://dx.doi.org/10.1002/ccr3.3933 | DOI Listing |
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.
View Article and Find Full Text PDFRelapsing polychondritis (RP) is a rare immune-mediated systemic inflammatory disease with diverse clinical manifestations. Independent involvement of the respiratory system in RP is uncommon. In the event of respiratory involvement as the initial airway-only manifestation, the diagnosis of RP is challenging and might be delayed, and patients with respiratory involvement exhibit a poor prognosis.
View Article and Find Full Text PDFZhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi
November 2024
Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology Head and Neck Surgery, National Center for Children's Health(NCCH), Beijing100045, China.
To investigate the pathogenesis, clinical signs and diagnosing procedures of relapsing polychondritis(RP) in children with airway involvement. The medical history, clinical symptoms, physical examination, electronic laryngoscopy and imaging findings of six patients were retrospectively analyzed. The patients diagnosed as relapsing polychondritis with involving the airway from January 2018 to December 2021 were in our hospital.
View Article and Find Full Text PDFRev Prat
November 2024
Service de médecine interne 2, centre de référence des maladies auto-immunes et autoinflammatoires systémiques rares de l'adulte d'Île-de-France, Centre et Martinique, hôpital de La Pitié-Salpêtrière, AP-HP, Paris, France. Association francophone contre la polychondrite chronique atrophiante (AFPCA).
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