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目的探讨胰腺外少见部位IgG4相关性疾病(IgG4-RD)的影像表现。方法回顾性分析21例确诊IgG4-RD患者临床和影像资料,包括CT、MRI及经内镜逆行胰胆管造影(ERCP)检查。20例行CT检查,11例行MRI检查,6例行ERCP检查。重点观察胰腺外少见部位IgG4-RD的受累情况和影像表现。结果 IgG4相关性胰腺炎患者13例,均伴有胰腺外组织器官受累;仅表现为胰腺外组织器官受累者8例。受累器官为1~5个,其中4例1个器官受累,5例2个器官受累,3例3个器官受累,4例4个器官受累,5例5个器官受累。胰腺外常见受累部位是:胆管受累13例,淋巴结肿大11例。少见受累部位是:肺部受累7例,表现为肺内结节型、磨玻璃结节型、肺泡间质型及支气管血管束增厚型4种类型;肾脏受累6例,CT表现为双肾多发低密度结节,呈延迟强化,MRI检查T_2WI呈特征性稍低信号,DWI稍高信号;胆囊受累5例,表现为胆囊壁弥漫性增厚;2例腹膜后纤维化和2例肠系膜受累表现为软组织肿块形成并包绕腹主动脉和肠系膜动脉;肾盂输尿管、唾液腺、鼻咽和硬脊膜受累各1例。结论 IgG4-RD的影像表现通常为受累部位弥漫或局限性肿大,增强扫描呈延迟强化。了解胰腺外少见部位IgG4-RD的临床及影像表现有助于该类疾病的鉴别诊断,从而选择合适的治疗方案。
Objective To investigate the imaging findings of IgG4-related diseases (IgG4-RD) in the rare parts of the pancreas. Methods The clinical and imaging data of 21 patients diagnosed with IgG4-RD were analyzed retrospectively, including CT, MRI and endoscopic retrograde cholangiopancreatography (ERCP). 20 cases underwent CT examination, 11 cases underwent MRI examination and 6 cases underwent ERCP examination. Focus on the rare parts of the pancreas outside the IgG4-RD involvement and imaging findings. Results Thirteen patients with IgG4-related pancreatitis were associated with extrapancreatic tissues and organs, and only 8 cases were found with involvement of extra-pancreatic tissues and organs. The affected organs ranged from 1 to 5, of which 4 were involved in 1 organ, 5 in 2 organs, 3 in 3 organs, 4 in 4 organs and 5 in 5 organs. Pancreatic involvement sites are: bile duct involvement in 13 cases, 11 cases of lymphadenopathy. The most common sites were: lung involvement in 7 cases, manifested as pulmonary nodules, ground glass nodules, alveolar interstitial and bronchial vascular bundle thickening type 4; kidney involvement in 6 cases, CT showed renal Multiple low-density nodules, delayed enhancement, T_2WI MRI showed slightly lower signal characteristic, DWI slightly higher signal; gallbladder involvement in 5 cases, showed diffuse thickening of the gallbladder wall; 2 cases of retroperitoneal fibrosis and 2 cases of mesenteric involvement Manifested as soft tissue mass formed and wrapped around the abdominal aorta and mesenteric artery; ureter, salivary gland, nasopharynx and dura impaired in 1 case. Conclusions The imaging manifestations of IgG4-RD are often diffuse or localized enlargement of the affected area, delayed enhancement enhanced scan. To understand the clinical and imaging manifestations of IgG4-RD in the rare parts of the pancreas is helpful in the differential diagnosis of these diseases so as to select the appropriate treatment plan.