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嗜酸性筋膜炎的临床、影像特征及误诊

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目的 分析嗜酸性筋膜炎(EF)的临床特征、影像学表现及其误诊原因,加强对该病临床特征、诊断要点及鉴别诊断的认识。方法 回顾性分析2018年10月~2023年3月安徽医科大学第二附属医院风湿免疫科收治的11例EF患者,其中男7例,女4例,发病年龄16~71岁。对其一般资料、临床表现、影像学检查、病理资料、治疗等情况进行分析。结果 11例EF患者的临床表现包括:皮下肿胀/紧硬(90。9%)、关节痛/炎(63。6%)、棍棒感(63。6%)、色素沉着(45。5%)、祈祷手(36。36%)、沟壑征(18。18%)、肌痛肌无力(9。09%)等。11例患者影像学检查均可见肌肉MRI检查受累肌筋膜增厚,1例发现肌肉受累异常信号且活检病理提示合并肌肉受累。11例患者行全层皮肤及肌肉活检,病理多可见筋膜增厚,伴慢性炎性反应、纤维素样变性、淋巴细胞或浆细胞浸润,7例见嗜酸粒细胞浸润。其中10例行骨髓穿刺活检,7例有嗜酸性粒细胞增生骨髓象,均未见血液系统肿瘤。结论 EF是一种罕见的结缔组织病,临床特征、影像学及病理学表现具有许多相似性,易误诊,深入了解临床、影像特征,分析误诊原因,避免误诊误治。
Clinical and imaging features and misdiagnosis of eosinophilic fasciitis
Objective To analyze the clinical features,imaging manifestations and misdiagnosis causes of eosinophilic fasciitis(EF),and to strengthen the understandings of clinical features,diagnostic key points and differential diagnosis of EF.Methods A retrospective analysis was performed on eleven EF patients admitted to Department of Rheumatology and Immunology of the Second Affiliated Hospital of Anhui Medical University from October 2018 to March 2023,including seven males and four females,with the age of onset ranging from 16 to 71 years old,and their demographics,clinical manifestations,imaging examination,pathological data,and treatment were analyzed.Results Clinical manifestations included subcutaneous swelling/stiffness(90.9%),arthralgia/inflammation(63.6%),sticky sensation(63.6%),pigmentation(45.5%),Prayer hand(36.36%),gully sign(18.18%),myalgia and muscle weakness(9.09%),etc.Imaging examination of eleven patients showed muscle MRI examination of the affected myofascial thickening,and one case found abnormal signal of muscle involvement and biopsy pathology suggested muscle involvement.Full-thickness skin and muscle biopsies were performed in eleven patients,and fascial thickening with chronic inflammation,fibrinoid degeneration,lymphocytic or plasma cell infiltration was mostly seen in pathology,and eosinophilic infiltration was seen in seven cases.Among them,ten cases underwent bone marrow biopsy,and seven cases had eosinophilic myelogram,and no hematologic tumors were found.Conclusion Eosinophilic fasciitis is a rare connective tissue disease,with many similarities in clinical features,imaging and pathological manifestations,and it is easy to be misdiagnosed.In-depth understanding of clinical and imaging features,analysis of causes of misdiagnosis can avoid misdiagnosis and mistreatment.

eosinophilic fasciitisclinical manifestationsimaging featurespathologymisdiagnosis

徐舟舟、赵红

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安徽医科大学第二附属医院风湿免疫科,安徽 合肥 230000

嗜酸性筋膜炎 临床表现 影像特征 病理 误诊

安徽省卫生健康委员会基金安徽医科大学第二附属医院转化医学研究所科研基金

AHWJ2023BAb200092022ZHYJ11

2024

分子影像学杂志
南方医科大学

分子影像学杂志

CSTPCD
ISSN:1674-4500
年,卷(期):2024.47(8)