Results 191 to 200 of about 391,559 (209)

Pathogenic UBA1 variants associated with VEXAS syndrome in Japanese patients with relapsing polychondritis

Annals of the Rheumatic Diseases, 2021
Objectives To determine clinical and genetic features of individuals with relapsing polychondritis (RP) likely caused by pathogenic somatic variants in ubiquitin-like modifier activating enzyme 1 (UBA1).
N. Tsuchida, Y. Kunishita, Yuri Uchiyama, Y. Kirino, M. Enaka, Y. Yamaguchi, M. Taguri, S. Yamanaka, Kaoru Takase-Minegishi, R. Yoshimi, S. Fujii, H. Nakajima, N. Matsumoto   +12 more
semanticscholar   +1 more source

Biologic therapy in relapsing polychondritis: navigating between options

Expert Opinion on Biological Therapy, 2022
Introduction Relapsing polychondritis (RP) is a rare systemic inflammatory disease of unknown etiology, primarily affecting cartilaginous tissue and proteoglycan-rich structures. Clinical manifestations vary from mild symptoms to occasional organ or life-
R. Padoan, D. Campaniello, L. Iorio, A. Doria, F. Schiavon   +4 more
semanticscholar   +1 more source

Tocilizumab in VEXAS relapsing polychondritis: a single-center pilot study in Japan

Annals of the Rheumatic Diseases, 2021
Recently, a rare severe autoinflammatory disease vacuoles, E1 enzyme, Xlinked, autoinflammatory, somatic (VEXAS) syndrome caused by somatic variants in the UBA1 gene was discovered. We reported the clinical features of eight relapsing polychondritis (RP)
Y. Kirino, Kaoru Takase-Minegishi, N. Tsuchida, Lisa Hirahara, Y. Kunishita, R. Yoshimi, H. Nakajima   +6 more
semanticscholar   +1 more source

Clinical analysis of relapsing polychondritis with airway involvement

Journal of Laryngology and Otology, 2022
Objective To identify the clinical characteristics, treatment, and prognosis of relapsing polychondritis patients with airway involvement. Methods Twenty-eight patients with relapsing polychondritis, hospitalised in the First Hospital of Shanxi Medical ...
Songyu Zhai, Ruyan Guo, Chen Zhang, Chunming Zhang, Hongyu Yin, Binquan Wang, Shuxin Wen   +6 more
semanticscholar   +1 more source

Relapsing polychondritis: state-of-the-art review with three case presentations

Postgraduate medicine, 2021
Background Relapsing polychondritis (RPC) is a complex immune-mediated systemic disease affecting cartilaginous tissue and proteoglycan-rich organs. The most common and earliest clinical features are intermittent inflammation involving the auricular and ...
B. Grygiel-Górniak, H. Tariq, Jacob Mitchell, Azad Mohammed, W. Samborski   +4 more
semanticscholar   +1 more source

Characteristics and Clinical Outcomes of 295 Patients With Relapsing Polychondritis

Journal of Rheumatology, 2021
Objective This study analyzes the clinical features of Chinese patients with relapsing polychondritis (RP). Methods The clinical data of 295 patients with RP at Beijing Tongren Hospital were retrospectively analyzed.
Nan Chen, Yi Zheng
semanticscholar   +1 more source

Relapsing polychondritis associated with heart block.

European Review for Medical and Pharmacological Sciences, 2021
OBJECTIVE The present article aims at describing a rare case of an RP patient who evolved with heart block and was successfully treated with corticoid pulse therapy, without the need for pacemaker insertion.
J. D. de Carvalho, L. C. Behrmann Martins, A. F. Cardoso, Y. Shoenfeld   +3 more
semanticscholar   +1 more source

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Annals of Otology, Rhinology and Laryngology, 2021
Objectives: To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods: Case report and literature review.
Özge Akdoğan, Smirnov Exilus, B. Ward, J. McArthur, C. D. Della Santina, J. Carey   +5 more
semanticscholar   +1 more source

Sustained Remission with Tocilizumab in Refractory Relapsing Polychondritis with Ocular Involvement: A Case Series

Ocular immunology and inflammation, 2020
Objective: Describe our experience with tocilizumab in the treatment of refractory relapsing polychondritis with ocular involvement. Methods: Retrospective consecutive interventional case series that included all patients that received tocilizumab for ...
R. Farhat, G. Clavel, D. Villeneuve, Y. Abdelmassih, M. Sahyoun, E. Gabison, T. Sené, I. Cochereau, C. Titah   +8 more
semanticscholar   +1 more source

Absolute reduction of peripheral regulatory T cell in patients with relapsing polychondritis.

Clinical and Experimental Rheumatology, 2020
OBJECTIVES Although relapsing polychondritis (RP) is considered as an immune-mediated systemic disease, the levels of peripheral lymphocyte subpopulations are rarely studied in patients with RP.
F. Hu, Jia Wang, Sheng-Xiao Zhang, R. Su, N. Yan, Chong Gao, Xiaofeng Li, Caihong Wang   +7 more
semanticscholar   +1 more source