Idiopathic retroperitoneal fibrosis associated with IgG4-positive-plasmacyte infiltrations and idiopathic chronic pancreatitis
Idiopathic retroperitoneal fibrosis associated with IgG4-positive-plasmacyte infiltrations and idiopathic chronic pancreatitis
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DOI：
10.1111/j.1442-2042.2006.01568.x
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发表时间：
2006-11-01
影响因子：
2.6
通讯作者：
Hara, Noboru
中科院分区：
文献类型：
--
作者：
Miyajima, Norio;Koike, Hiroshi;Hara, Noboru
特发性腹膜后纤维化（IRPF）是一种炎症性纤维硬化性疾病，通过输尿管梗阻导致肾功能衰竭。特发性慢性胰腺炎与显着的炎性浸润最近被称为自身免疫性胰腺炎（AIP），浸润浆细胞携带免疫球蛋白-γ 4型（IgG 4）是其发病机制相关。本文描述的情况下，IRPF与亚临床胰腺炎，最有可能是AIP在一个70岁的男子。活检标本的腹膜后假瘤显示了显着的淋巴浆细胞浸润与致密纤维化。浸润浆细胞的抗IgG 4抗体的免疫反应。随后的全身检查显示血清IgG 4水平极高，胰腺炎与AIP一致。口服类固醇给药后，血清IgG 4水平正常化，但假瘤的外观没有改变。一些AIP病例与特发性纤维硬化性疾病（包括IRPF）相关，但很少提供IgG 4相关IRPF的组织学证据。
Idiopathic retroperitoneal fibrosis (IRPF) is an inflammatory fibrosclerosing condition, leading to renal failure by obstruction of the ureters. Idiopathic chronic pancreatitis associated with marked inflammatory infiltrates has recently been referred to as autoimmune pancreatitis (AIP), and infiltrating plasmacytes carrying immunoglobulin-gamma type 4 (IgG4) are relevant to its pathogenesis. The case is described herein of IRPF associated with subclinical pancreatitis that was most probably AIP in a 70-year-old man. Biopsy specimens of the retroperitoneal pseudotumor revealed a marked lymphoplasmacytic infiltration with dense fibrosis. Infiltrating plasma cells were immunoreactive for anti-IgG4 antibodies. Subsequent systemic examinations showed an extremely elevated serum IgG4 level and pancreatitis concordant with AIP. Following oral steroid administration, the serum IgG4 level normalized, although the appearance of the pseudotumor did not alter. Some AIP cases have been associated with idiopathic fibrosclerosing disorders including IRPF, but histological evidence of IgG4-related IRPF has rarely been provided.