Sangha Brar Jaspreet Singh, Gupta Saurav, Haja Mohideen Salahudeen M, Liauw Lishya, Lath Narayan
Department of Diagnostic Radiology, Ng Teng Fong General Hospital, Jurong East, Singapore.
Diagnostic Radiology Service, Singapore General Hospital, Outram Road, Singapore.
Diagn Interv Radiol. 2018 Mar-Apr;24(2):83-88. doi: 10.5152/dir.2018.17319.
The association between immunoglobulin IgG4 and autoimmune pancreatitis was first shown in 2001. Since then many previously established fibrosclerotic diseases demonstrating synchronous or metachronous multiorgan involvement have been included within the ambit of IgG4-related disease. Diagnostic criteria have been proposed involving 1) serum IgG4 level elevated beyond 135 mg/dL, 2) IgG4+ to IgG+ plasma cell ratio > 40% and >10 IgG4+ cells per high power field of biopsy sample and 3) a constellation of imaging features which involve a variety of organ systems. We present a pictorial essay demonstrating the spectrum of imaging findings for IgG4-related disease, including dacryosialadenitis, variety of renal lesions, tumefactive thickening of the extraocular muscles and orbital nerve, sclerosing cholangitis, and type I pancreatitis. Imaging plays an important role in diagnosis, screening for multiorgan involvement, and follow-up of the disease.
免疫球蛋白IgG4与自身免疫性胰腺炎之间的关联于2001年首次被发现。从那时起，许多先前已确定的表现为同步或异时多器官受累的纤维硬化性疾病已被纳入IgG4相关疾病的范畴。已提出诊断标准，包括：1）血清IgG4水平升高超过135mg/dL；2）活检样本每高倍视野中IgG4+与IgG+浆细胞比例>40%且IgG4+细胞>10个；3）一系列涉及多种器官系统的影像学特征。我们展示了一组图片文章，呈现了IgG4相关疾病的影像学表现谱，包括泪腺唾液腺炎、各种肾脏病变、眼外肌和眶神经的肿块样增厚、硬化性胆管炎以及I型胰腺炎。影像学在该疾病的诊断、多器官受累筛查及随访中发挥着重要作用。
