IgG4相关疾病的影像特征及疗效监测

目的：探讨IgG4相关疾病的影像学特征及激素治疗后疗效的评估。方法收集13例经病理证实的IgG4相关疾病的影像资料,均行CT检查,其中5例同时行MR检查,6例患者有治疗前及治疗后3~6月的完整影像资料。分析病灶的形态、分布、影像特征以及治疗后影像变化。结果13例中1例病灶位于泪腺,1例位于颌下腺,1例位于胆道,3例位于胰腺,4例位于肾脏,2例位于腹膜后,1例位于肺。其中2例病变累及多部位,包括腹膜后、胰腺及肾脏等。所有受累脏器呈弥漫性肿胀或局部软组织肿块,CT平扫呈低密度,T2WI上呈低信号,增强后轻度均匀性强化。6例患者经激素治疗后病灶缩小,T2WI信号有增高表现。结论受累脏器肿胀,CT平扫低密度以及T2WI低信号,轻度均匀强化是IgG4相关疾病的影像学特征,CT及MR在疾病的诊断及激素治疗后疗效的评估方面具有重要的临床应用价值。

Imaging features and curative effect monitoring of IgG4-related disease

Objective To explore imaging features of IgG4-related diseases (IgG4-RD)and to evaluate curative effect after treatment with steroid.Methods Imaging data were reviewed retrospectively in thirteen patients with pathologically proved IgG4-RD.CT scans were performed in all patients,while MR scans in five of them.Six patients were examined before and after steroid treatment in 3-6 months. Imaging data were analyzed with regard to the shape,distribution and characteristics of the lesions.The image changes between pre-and post-treatment with steroid were analyzed as well.Results The IgG4-RD occurred in the lacrimal gland in 1 case,submandibular glands in 1 case,biliary duct in 1 case,pancreas in 3 cases,kidney in 4 cases,retroperitoneum in 2 cases and lung in 1 case.Multiple organs including the retroperitoneum,kidney and pancreas were involved in two patients.Diffusely swelling organ or soft tissue mass, which manifested as low density on CT and low signal intensity on T2-weighted images on MR,were characteristics of IgG4-RD.All lesions mildly enhanced with contrast media administration.The lesions decreased in size accompanied by raised signal intensity on T2 WI after treatment with steroid in six patients.Conclusion Diffusely swelling organ,low density and low signal intensity on T2 WI with mildly homogenous enhancement are characteristics of IgG4-RD.CT and MR plays a vital role in diagnosis of IgG4-RD and monitoring the curative effect with steroid treatment.