ABSTRACT

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 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.

Method

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.

Result

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.

Conclusion

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.

中文翻译：

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复发性多软骨炎：三个病例介绍的最新回顾

摘要

背景

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多糖的器官。最常见和最早的临床特征是涉及耳廓和鼻腔区域的间歇性炎症，尽管所有软骨类型都可能受到影响。rpc 的危及生命的影响涉及气管支气管树和心脏结缔成分。Rpc 在其他自身免疫合并症中难以识别；诊断通常被延迟，并且基于非特异性临床症状，实验室帮助和调查有限。药物可能会有所不同，包括类固醇、免疫抑制剂和生物制剂，包括抗 tnf α 拮抗剂药物。

方法

通过使用 PubMed 和 medline 数据库对 1976 年至 2019 年间发表的电子文献进行广泛研究，获得了关于 rpc 的最新病因、临床症状、诊断和治疗的信息。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”和“复发性多软骨炎的治疗”。概述和报告 rpc 临床表现和治疗的英文发表文章最终符合纳入标准。未报告上述内容并报告其他软骨疾病的文章符合排除标准。

结果

利用对 RPC 研究关键领域工作的广泛概述，本综述旨在从病理生理学、诊断和管理的各个方面进一步探索和教育该疾病的治疗方法。

结论

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，并根据每个病例的疾病严重程度来确定。推进的最佳方式是继续从参考中心共享 RPC 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当前对 RPC 的了解，这不仅会改进治疗，还会改进诊断方法，包括成像和生物标志物。