An Yu-Qiong, Ma Ning, Liu Yong
Heart Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.
Department of Ultrasound, Beijing-Shijitan Hospital, Capital Medical University, Beijing 100038, China.
World J Clin Cases. 2022 Mar 16;10(8):2543-2549. doi: 10.12998/wjcc.v10.i8.2543.
IgG4-related disease (IgG4-RD), an immune-mediated chronic progressive fibroinflammatory disease, can affect the functions of several organs. Some common characteristics can be observed in different IgG4-RDs, such as higher prevalence in middle-aged and elderly male patients, raised serum IgG4 levels, abundant infiltration of IgG4-positive cells and fibrosis, diffuse or localized swelling of the affected organs, and good response to glucocorticoids treatment.
A 72-year-old man complained of left upper abdominal pain 3 mo ago, and he was diagnosed with acute onset of chronic cholecystitis and acute pancreatitis in the local hospital. Pain improved after relevant treatment. Several days ago, his abdominal pain worsened, and he was admitted to our hospital for further treatment. Doppler ultrasound showed that the pancreas presented with sausage-like swelling and the parenchymal echo was diffusely reduced. Gallbladder volume was increased, while the wall was rough and thickened with bilateral signs. Furthermore, the left submandibular gland was enlarged, accompanied with significantly increased blood flow signals. Finally, we found that the adventitia of the abdominal aorta and right iliac artery was thickened locally. Serum IgG4 was elevated to 12600 mg/L. Therefore, the patient was diagnosed with IgG4-RD. After treatment with methylprednisolone, he had an uneventful course and was discharged in good condition.
IgG4-RD can involve almost any organs. Ultrasound has a significant role in timely and accurately diagnosis.
IgG4相关性疾病（IgG4-RD）是一种免疫介导的慢性进行性纤维炎性疾病，可影响多个器官的功能。不同的IgG4-RD具有一些共同特征，如在中老年男性患者中患病率较高、血清IgG4水平升高、IgG4阳性细胞大量浸润及纤维化、受累器官弥漫性或局限性肿胀，以及对糖皮质激素治疗反应良好。
一名72岁男性3个月前出现左上腹疼痛，在当地医院被诊断为慢性胆囊炎急性发作和急性胰腺炎。经过相关治疗后疼痛缓解。数天前，他的腹痛加重，遂入住我院进一步治疗。多普勒超声显示胰腺呈腊肠样肿胀，实质回声弥漫性减低。胆囊体积增大，胆囊壁粗糙增厚，呈双边征。此外，左下颌下腺肿大，血流信号明显增多。最后，我们发现腹主动脉和右髂动脉外膜局部增厚。血清IgG4升高至12600mg/L。因此，该患者被诊断为IgG4-RD。经甲泼尼龙治疗后，患者病情平稳，康复出院。
IgG4-RD可累及几乎任何器官。超声在及时准确诊断中具有重要作用。
