Department of Radiology, The Eastern Hospital of the First Affiliated Hospital, Sun Yat-Sen University, No.183 Huangpu Eastern Road, Guangzhou, 510700, Guangdong, People's Republic of China.
Department of Radiology, The First Affiliated Hospital of Sun Yat-Sen University, No.58 Zhongshan Road 2, Guangzhou, 510080, Guangdong, People's Republic of China.
Abdom Radiol (NY). 2020 Jun;45(6):1915-1921. doi: 10.1007/s00261-020-02477-8.
To assess the clinical and imaging features of IgG4-RKD for understanding and diagnosis of this disease.
CT and MR images of 34 patients with IgG4-RKD were retrospectively analyzed by two radiologists in consensus.
The serum IgG4 level was found being increased in all patients. Renal involvement was bilateral (24/34, 70.6%) or unilateral (10/34,29.4%), multiple (29/34, 85.3%) or solitary (5/34, 14.7%). The lesions were wedge-shaped (21) or mass-like (4) in the renal parenchyma, whereas diffusely decreased renal density was noted in 2 patients. All lesions showed progressive contrast enhancement. The 4 mass-like lesions were misdiagnosed as renal malignancy. In 15 patients with follow-up imaging examinations, the number and size of renal lesions decreased after oral hormone treatment. The serum IgG4 levels were significantly decreased after therapy in all patients.
IgG4-RKD has various imaging appearances. Although the mass-like appearance mimics renal malignancy in some patients, progressive contrast enhancement in the lesion with elevated serum IgG4 suggests IgG4-RKD.
评估 IgG4-RKD 的临床和影像学特征，以帮助理解和诊断该病。
由 2 名放射科医生对 34 例 IgG4-RKD 患者的 CT 和 MR 图像进行回顾性分析。
所有患者的血清 IgG4 水平均升高。肾脏受累为双侧（24/34，70.6%）或单侧（10/34，29.4%），多发（29/34，85.3%）或单发（5/34，14.7%）。肾脏实质内病变呈楔形（21 例）或肿块样（4 例），2 例患者肾密度弥漫性降低。所有病变均呈渐进性对比增强。4 例肿块样病变误诊为肾恶性肿瘤。在 15 例有随访影像学检查的患者中，口服激素治疗后，肾脏病变的数量和大小均减少。所有患者治疗后血清 IgG4 水平均显著降低。
IgG4-RKD 的影像学表现多种多样。虽然某些患者的肿块样表现类似于肾恶性肿瘤，但病变呈渐进性对比增强且血清 IgG4 升高提示 IgG4-RKD。
