Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis
Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis
复制标题
DOI：
10.1007/s00535-006-1908-9
复制
发表时间：
2006-12-01
影响因子：
6.3
通讯作者：
Kawa, Shigeyuki
中科院分区：
文献类型：
--
作者：
Hamano, Hideaki;Arakura, Norikazu;Kawa, Shigeyuki
背景自身免疫性胰腺炎（Autoimmune pancreatitis，AAP）是一种独特的慢性胰腺炎，其特征是高血清IgG 4浓度和胰腺病变中大量携带IgG 4的浆细胞浸润，并且已报道其与多种胰腺外病变相关，导致我们假设系统性炎症性疾病的概念。为了证实这一点，我们阐明了这些胰腺外病变的确切分布，并提供了它们的全景图。方法.通过检查临床和实验室结果，确定了64例自身免疫性胰腺炎患者中5个胰外病变的频率、分布、临床特征和病理学。结果最常见的胰外病变是肝门淋巴结病（80.4%），其次是胰外胆管病变（73.9%），泪腺和唾液腺病变（39.1%），甲状腺功能减退症（22.2%）和腹膜后纤维化（12.5%）。没有患者具有所有五种类型的病变。发现有肺门淋巴结病或泪腺和唾液腺病变的患者的IgG 4水平显著高于无病变的患者（P分别为0.0042和0.0227）。发现有三处病变的患者的IgG 4水平显著高于无病变的患者，这表明有多处胰腺外病变的患者具有活动性疾病。与胰腺病变相似，胰腺外病变具有丰富的携带IgG 4的浆细胞浸润的特征性组织学发现，并且它们对皮质类固醇治疗反应良好。结论.自身免疫性胰腺炎是一种全身性炎症性疾病。此外，认识到这些特征性的发现将有助于这种疾病的正确诊断。
Background. Autoimmune pancreatitis is a unique form of chronic pancreatitis characterized by high serum IgG4 concentrations and abundant IgG4-bearing plasma cell infiltration in the pancreatic lesion, and it has been reported to be associated with a variety of extrapancreatic lesions, leading us to postulate the concept of a systemic inflammatory disease. To confirm this, we clarified the exact distribution of these extrapancreatic lesions and provide a panoramic view of them. Methods. The frequency, distribution, clinical characteristics, and pathology of five extrapancreatic lesions were determined in 64 patients with autoimmune pancreatitis by examining clinical and laboratory findings. Results. The most frequent extrapancreatic lesion was hilar lymphadenopathy (80.4%), followed by extrapancreatic bile duct lesions (73.9%), lachrymal and salivary gland lesions (39.1%), hypothyroidism (22.2%), and retroperitoneal fibrosis (12.5%). No patients had all five types of lesions. Patients with hilar lymphadenopathy or lachrymal and salivary gland lesions were found to have significantly higher IgG4 levels than those without (P = 0.0042 and 0.0227, respectively). Patients with three lesions were found to have significantly higher IgG4 levels than those with no lesion, suggesting that patients with multiple extrapancreatic lesions have active disease. Similar to pancreatic lesions, extrapancreatic lesions have a characteristic histological finding of abundant IgG4-bearing plasma cell infiltration, and they respond favorably to corticosteroid therapy. Conclusions. Autoimmune pancreatitis was recognized as a systemic inflammatory disease. Furthermore, recognition of these characteristic findings will aid in the correct diagnosis of this disease.