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伊朗复发性多软骨炎长期预后的多中心研究。

A multicenter study of long-term outcomes of relapsing polychondritis in Iran.

作者信息

Jafarpour Mehdi, Saberivand Maryam, Saemi Maryam, Sahebari Maryam, Seyedmardani Seyedmostafa, Salesi Mansour, Hosseinpoor Sarah, Faezi Tahereh, Esalatmanesh Kamal, Hajialilo Mehrzad, Kolahi Sousan, Myrfeizi Zahra, Khabbazi Alireza

机构信息

Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Golgasht St., Tabriz, Iran.

Rheumatic Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

出版信息

Sci Rep. 2024 Jul 17;14(1):16486. doi: 10.1038/s41598-024-67530-8.

DOI:10.1038/s41598-024-67530-8
PMID:39020004
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11255289/
Abstract

Relapsing polychondritis (RP) is a systemic immune mediated disease characterized by recurrent episodes of inflammation in various cartilage-rich areas. RP may cause extensive tissue destruction and is associated with significant morbidity and mortality. In this multicenter study, we considered the remission status and long-term outcomes of RP in patients who were followed-up in six referral rheumatology centers in Iran. Outcomes of disease was assessed by remission status and RP induced damage. A total of 29 patients with RP were examined for enrollment in the study, and 26 patients with a minimum follow-up period of 6 months were included in the RP outcome analysis. Median time to control of symptoms and sustained remission were 5 and 23 weeks, respectively. Prednisolone was discontinued in 8 (30.8%) patients and medication-free remission was achieved in 7 (23.1%) patients. Regarding the disease course, 34.6% of patients had a relapsing-remitting course, 42.3% had a monophasic course, and 23.1% had an always-active course. Despite extensive treatment with immunosuppressive medications, RP induced damage was developed in 21 (80.8%) patients. Ear deformity and osteoporosis were the most common RP induced damage. Long-term remission and medications-free remission in RP is accessible. However, RP related damage occur in majority of patients.

摘要

复发性多软骨炎(RP)是一种全身性免疫介导疾病,其特征为富含软骨的各个部位反复发生炎症发作。RP可导致广泛的组织破坏,并与显著的发病率和死亡率相关。在这项多中心研究中,我们考察了在伊朗六个转诊风湿病中心接受随访的RP患者的缓解状态和长期预后。通过缓解状态和RP所致损害评估疾病的预后。共有29例RP患者接受研究入组检查,26例随访期至少6个月的患者纳入RP预后分析。症状得到控制的中位时间和持续缓解时间分别为5周和23周。8例(30.8%)患者停用泼尼松龙,7例(23.1%)患者实现无药缓解。就病程而言,34.6%的患者为复发缓解型病程,42.3%为单相病程,23.1%为持续活动型病程。尽管使用免疫抑制药物进行了广泛治疗,但21例(80.8%)患者仍出现了RP所致损害。耳部畸形和骨质疏松是最常见的RP所致损害。RP患者可实现长期缓解和无药缓解。然而,大多数患者会出现与RP相关的损害。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/de95/11255289/6ee33f2c974d/41598_2024_67530_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/de95/11255289/6ee33f2c974d/41598_2024_67530_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/de95/11255289/6ee33f2c974d/41598_2024_67530_Fig1_HTML.jpg

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