特发性纤维性纵隔炎：影像学表现谱，重点关注其与IgG4相关疾病的关联
Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease.
作者信息
Gorospe Luis, Ayala-Carbonero Ana María, Fernández-Méndez María Ángeles, Arrieta Paola, Muñoz-Molina Gemma María, Cabañero-Sánchez Alberto, Mañas-Baena Eva
机构信息
Department of Radiology, Hospital Universitario Ramón y Cajal, 28034 Madrid, Spain.
Department of Radiology, Hospital Universitario Ramón y Cajal, 28034 Madrid, Spain.
出版信息
Clin Imaging. 2015 Nov-Dec;39(6):993-9. doi: 10.1016/j.clinimag.2015.07.008. Epub 2015 Jul 16.
PURPOSE
The purpose of this study was to review the clinicoradiological features of idiopathic fibrosing mediastinitis (FM).
METHODS
The records of six patients with idiopathic FM were retrospectively reviewed.
RESULTS
The most common presenting symptom was cough. There were three localized forms and three diffuse forms. Definitive histological diagnosis was most commonly made with mediastinoscopy. Interestingly, three of our patients had disease manifestations of IgG4-related disease (IgG4-RD) outside the mediastinum.
CONCLUSION
The imaging findings of idiopathic FM vary depending on the involved mediastinal structures. Therapeutic options include medical treatment, stenting, or surgery. Radiologists should pay attention to the possible association of idiopathic FM with extrathoracic manifestations of the IgG4-RD spectrum.
目的
本研究旨在回顾特发性纵隔纤维化（FM）的临床放射学特征。
方法
回顾性分析6例特发性FM患者的病历。
结果
最常见的首发症状是咳嗽。有3例局限性形式和3例弥漫性形式。确诊的组织学诊断最常通过纵隔镜检查做出。有趣的是，我们的3例患者在纵隔外有IgG4相关疾病（IgG4-RD）的疾病表现。
结论
特发性FM的影像学表现因受累纵隔结构而异。治疗选择包括药物治疗、支架置入或手术。放射科医生应注意特发性FM与IgG4-RD谱胸外表现的可能关联。
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