关键词: Relapsing polychondritis auricular chondritis diagnosis etiology polychondritis relapsing/diagnosis polychondritis relapsing/drug therapy polychondritis relapsing/etiology treatment

来  源:   DOI:10.1080/00325481.2021.1979873

Abstract:
UNASSIGNED: 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 nasal regions, although all cartilage types can be potentially affected. The life-threatening effects of rpc involve the tracheobronchial tree and cardiac connective components. Rpc is difficult to identify among other autoimmune comorbidities; diagnosis is usually delayed and based on nonspecific clinical symptoms with limited laboratory aid and investigations. Medications can vary, from steroids, immunosuppressants, and biologics, including anti-tnf alpha antagonist drugs.
UNASSIGNED: Information on updated etiology, clinical symptoms, diagnosis, and treatment of rpc has been obtained via extensive research of electronic literature published between 1976 and 2019 using PubMed and medline databases. English was the language of use. Search inputs included \'relapsing polychondritis,\' \'polychondritis,\' \'relapsing polychondritis symptoms,\' and \'treatment of relapsing polychondritis.\' Published articles in English that outlined and reported rpc\'s clinical manifestations and treatment ultimately met the inclusion criteria. Articles that failed to report the above and reported on other cartilaginous diseases met the exclusion criteria.
UNASSIGNED: Utilizing an extensive overview of work undertaken in critical areas of RPC research, this review intends to further explore and educate the approach to this disease in all dimensions from pathophysiology, diagnosis, and management.
UNASSIGNED: RPC is a rare multi-systemic autoimmune disease and possibly fatal. The management remains empiric and is identified based on the severity of the disease per case. The optimal way to advance is to continue sharing data on RPC from reference centers; furthermore, clinical trials in randomized control groups must provide evidence-based treatment and management. Acquiring such information will refine the current knowledge of RPC, which will improve not only treatment but also diagnostic methods, including imaging and biological markers.
摘要:
复发性多软骨炎(RPC)是一种复杂的免疫介导的全身性疾病,影响软骨组织和富含蛋白聚糖的器官。最常见和最早的临床特征是涉及耳部和鼻部的间歇性炎症。尽管所有软骨类型都可能受到影响。rpc的危及生命的作用涉及气管支气管树和心脏结缔组织成分。在其他自身免疫性合并症中,Rpc很难识别;诊断通常会延迟,并且基于非特异性临床症状,而实验室辅助和检查有限。药物可以有所不同,从类固醇,免疫抑制剂,和生物制品,包括抗tnfα拮抗剂药物。
有关最新病因的信息,临床症状,诊断,rpc的治疗是通过使用PubMed和medline数据库对1976年至2019年之间发表的电子文献进行的广泛研究获得的。英语是使用的语言。搜索输入包括复发性多软骨炎,\'\'多软骨炎,\'\'复发性多软骨炎症状,复发性多软骨炎的治疗。以英文发表的文章概述并报告了rpc的临床表现和治疗最终符合纳入标准。未能报告上述内容并报告其他软骨疾病的文章符合排除标准。
利用在RPC研究的关键领域开展的工作的广泛概述,这篇综述旨在从病理生理学的各个方面进一步探索和教育这种疾病的方法,诊断,和管理。
RPC是一种罕见的多系统自身免疫性疾病,可能致命。管理仍然是经验性的,并根据每例疾病的严重程度进行识别。推进的最佳方式是继续从参考中心共享RPC上的数据;此外,随机对照组的临床试验必须提供循证治疗和管理.获取这些信息将完善RPC的当前知识,这不仅会改善治疗,还会改善诊断方法,包括成像和生物标记。
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