IgG4相关性疾病的影像表现.ppt

THANK YOU IgG ；IgA ；IgM ；IgD ；IgE 中老年易患，男性多见 发热、CRP升高 58例病人 * 在IgG4-RD国际诊治指南专家共识中，其综合诊断标准包括三条：临床检查显示1个或多个器官特征性地弥漫性/局限性肿大或肿块形成;血液学检查IgG4升高(135mg/dl);组织学检查显示大量淋巴细胞和浆细胞浸润，伴纤维化，且组织中浸润的IgG4+浆细胞与浆细胞的比值40%，在高倍镜视野下IgG4+浆细胞10个。三者皆有即可确诊，两者存在为可疑或可能。 * Figure 3. Different patterns of autoimmune pancreatitis. (a) Drawing shows the normal appearances of the pancreas, pancreatic duct, and distal common bile duct. (b) Drawing shows diffuse autoimmune pancreatitis, which demonstrates irregular narrowing of the entire pancreatic duct, with a halo around the gland and focal, irregular narrowing of the intrapancreatic portion of the common bile duct. (c) Drawing shows focal autoimmune pancreatitis, which affects the head of the pancreas, with localized stenosis of the pancreatic duct and intrapancreatic portion of the common bile duct and mild upstream pancreatic duct and bile duct dilatation. (d) Drawing shows multifocal autoimmune pancreatitis, which demonstrates segmental narrowing in the affected segments of the pancreatic and bile ducts. * Diffuse autoimmune pancreatitis in a 75-year-old man. (a) Contrast-enhanced CT image shows a mildly heterogeneous pancreas surrounded by a hypoattenuating halo. (b) Follow-up CT image obtained 8 months later, after steroid treatment, shows the pancreas, which appears mildly atrophic with no halo. Thickening and enhancement of the common bile duct (arrow) are seen. * Venous phase oblique axial (a) and coronal (b) reformatted CT images show focal enlargement of the pancreatic tail with a distinct area of decreased attenuation (arrows). Minimal stranding is seen around the enlarged pancreatic tail. * CT增强扫描（早期）的IgG4相关的血管病变在55岁的老人。）一个椎旁软组织肿块（箭头）也被诊断出；在穿刺活检，确诊为IgG4相关疾病。 IgG4相关性胰腺炎 图c.由于炎症，病变相对于胰腺实质呈高信号 图d.主胰腺管的狭窄及上游的轻度扩张，碎冰锥征 IgG4相关性胰腺炎与其他疾病的鉴别 胰腺钙化和假性囊肿形成通常出现在慢性胰腺炎, 严重的胰周渗出是急性胰腺炎的一个典型特征,这些在自身免疫性胰腺炎中是罕见的。 尽管一些自身免疫性胰腺炎和胰脏癌同时发生的病例已报告, 但是还没有数据支持这种IgG4-相关性疾病使恶性肿瘤风险增加的观点。 2010年 AlP诊断标准国际共识 诊断依据包括影像学(细分为胰腺实质影像学和胰管影像学)、血清