头颈部非腺体IgG4相关性疾病的影像分析

Imaging analysis of IgG4-related disease involving non-glandular parts of head and neck

目的探讨头颈部非腺体IgG4相关性疾病(IgG4-RD)的影像学特征,提高诊断水平。方法对14例我院确诊为IgG4-RD患者的临床资料和非腺体部位的影像表现进行回顾性分析。术前5例行MRI和CT扫描,5例行MRI扫描,4例行CT扫描。结果本组14例患者中,入院血清IgG4水平均大于1.35 g/L,病史1周至10余年。14例患者均出现非腺体部位受累并经活检病理确诊。影像学表现为鼻腔-鼻窦病灶为浸润性软组织肿块影(6例),可观察到骨质浸润破坏。周围神经受累表现为沿三叉神经分支和眶下神经走行的软组织影(3例),伴有眶下裂、眶下孔的扩大。局部淋巴结受累表现为颈部多发性淋巴结肿大(3例),密度/信号均匀。硬脑脊膜受累表现为硬脑脊膜弥漫性增厚(2例),伴有明显强化。结论IgG4-RD可原发或侵犯头颈部非腺体部位,鼻腔-鼻窦骨质改变、神经周生长和脑脊膜肥厚的表现具有一定的特征性,但确诊仍需结合临床及病理。

Objective To assess imaging features of immunoglobulin G4-related disease(IgG4-RD)in the non-glandular parts of head and neck.Methods Clinical data of 14 patients with IgG4-RD in our hospital were reviewed and preoperative MRI(10)and CT(9)features of pathologically confirmed non-glandular involvement were summarized.Results In all 14 patients with history ranging from 1 week to more than 10 years,the serum IgG4 level on admission was>1.35 g/L.Imaging appearance of 6 patients with paranasal sinus involvement included infiltrative soft tissue mass and bone destruction.In 3 patients with peripheral nerve involvement,soft tissue along trigeminal nerve branch and infraorbital nerve as well as enlarged infraorbital fissure and infraorbital foramen was shown on imaging.Local lymph node involvement in 3 patients presented as multiple enlarged cervical lymph nodes with well-defined margins and homogeneous CT density or MR signal intensity.Diffuse dura mater thickening with clear margins and obvious enhancement was depicted in 2 patients with dura mater involvement.Conclusion In patients with IgG4-RD,the imaging appearance of paranasal sinus bone change,perineural growth,and meningeal hypertrophy can aid the diagnosis of non-glandular involvement in the head and neck.