Suppr超能文献

一例表现为双侧耳软骨炎的未分化结缔组织病:一种罕见的临床关联。

A Case of Undifferentiated Connective Tissue Disease with Bilateral Auricular Polychondritis Manifestations: A Rare Clinical Association.

作者信息

Nigro Angelo, Santarcangelo Pasquale, Bonelli Antonio, Digregorio Serena, Nicoletti Giuseppe

机构信息

Department of Rheumatology of Lucania, UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy.

Department of Internal Medicine, "Madonna delle Grazie" Hospital, Matera, Italy.

出版信息

Am J Case Rep. 2025 May 8;26:e946827. doi: 10.12659/AJCR.946827.

Abstract

BACKGROUND Undifferentiated connective tissue disease (UCTD) is a heterogeneous autoimmune condition characterized by clinical features of connective tissue involvement without meeting the full classification criteria for a defined connective tissue disease (CTD). It often includes arthralgias, Raynaud's phenomenon, and serologic abnormalities. In rare cases, UCTD presents with features resembling relapsing polychondritis, raising questions about a possible overlap syndrome or a shared immunopathogenic mechanism. This case report describes an atypical presentation of UCTD with bilateral auricular inflammation and its therapeutic management. CASE REPORT A 45-year-old man diagnosed with UCTD initially presented with acral cyanosis and inflammatory arthralgias, which responded well to hydroxychloroquine and low-dose prednisone. Over time, he developed progressive bilateral auricular pain, erythema, and nodular swelling, mimicking polychondritis. In the absence of systemic features of relapsing polychondritis, this was considered an unusual manifestation within the UCTD spectrum rather than a distinct overlap syndrome. His symptoms improved significantly following an increase in corticosteroid therapy. CONCLUSIONS This case highlights the importance of recognizing atypical inflammatory manifestations in UCTD and adjusting treatment accordingly. The findings suggest auricular inflammation is part of the broader UCTD spectrum rather than a separate disease entity. These observations underscore the role of shared autoimmune pathways in connective tissue disorders and the need for individualized treatment approaches.

摘要

背景

未分化结缔组织病(UCTD)是一种异质性自身免疫性疾病,其特征为具有结缔组织受累的临床特征,但未达到明确结缔组织病(CTD)的完整分类标准。它通常包括关节痛、雷诺现象和血清学异常。在罕见情况下,UCTD会出现类似复发性多软骨炎的特征,这引发了关于可能的重叠综合征或共同免疫致病机制的问题。本病例报告描述了一例伴有双侧耳廓炎症的UCTD非典型表现及其治疗管理。

病例报告

一名45岁被诊断为UCTD的男性最初表现为肢端发绀和炎性关节痛,对羟氯喹和低剂量泼尼松反应良好。随着时间推移,他出现了进行性双侧耳廓疼痛、红斑和结节性肿胀,类似多软骨炎。由于缺乏复发性多软骨炎的全身特征,这被认为是UCTD范围内的一种不寻常表现,而非一种独特的重叠综合征。在增加皮质类固醇治疗后,他的症状明显改善。

结论

本病例突出了认识UCTD中非典型炎症表现并相应调整治疗的重要性。研究结果表明耳廓炎症是更广泛UCTD范围的一部分,而非一个单独的疾病实体。这些观察结果强调了自身免疫共同途径在结缔组织疾病中的作用以及个体化治疗方法的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5893/12070814/b69167ce379f/amjcaserep-26-e946827-g001.jpg

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验