Skorus Urszula, Kenig Jakub, Mastalerz Kinga
Uniwersytet Jagielloński Collegium Medicum Wydział Lekarski Klinika Chirurgii Ogólnej, Onkologicznej oraz Geriatrycznej, Uniwersytet Jagielloński Collegium Medicum, Kraków, Polska.
Pol Przegl Chir. 2018 Jun 13;90(4):41-45. doi: 10.5604/01.3001.0012.0976.
IgG4-related disease (IgG4-RD) is a newly recognised disorder of unknown etiology and pathogenesis, characterised by dense IgG4+ cells infiltration and fibrosis. IgG4-RD can affect various organs, but gastrointestinal tract involvement is rare. First case of isolated gastric IgG4-RD reported in polish population was diagnosed in our Clinic and became the reason for conducting a literature review.
A literature review was performed using PubMed database. Eight studies of isolated gastric IgG4-RD, published between 2011 and 2017, and a case diagnosed by the authors were included.
Three out of nine analysed patients had gastrointestinal complaints. In other cases lesions were detected accidentally. The majority of them were submucosal tumors while only one was a gastric ulcer. The most commonly affected was the stomach body. In all cases malignancy had been suspected, and the lesions were surgically removed. Diagnosis was based on the histopathology image and immunohistochemical staining. Only one patient had elevated IgG4 serum level. No case of recurrence or other organ involvement was reported.
IgG4-related disease may manifest as an isolated gastric lesion and should be taken in consideration in differential diagnosis. Making an ultimate diagnosis without histopathological specimen examination seems to be difficult and can lead to misdiagnosis followed by inappropriate treatment. IgG4-RD responds well to steroid therapy. However, on this matter further studies are needed.
IgG4相关性疾病（IgG4-RD）是一种病因和发病机制不明的新认识疾病，其特征为密集的IgG4+细胞浸润和纤维化。IgG4-RD可累及多个器官，但胃肠道受累罕见。我们诊所诊断出波兰人群中首例孤立性胃IgG4-RD，这成为进行文献综述的原因。
使用PubMed数据库进行文献综述。纳入了2011年至2017年间发表的8项关于孤立性胃IgG4-RD的研究以及作者诊断的1例病例。
9例分析患者中有3例有胃肠道症状。其他病例病变是偶然发现的。其中大多数是黏膜下肿瘤，只有1例是胃溃疡。最常受累的是胃体。所有病例均怀疑有恶性病变，病变均行手术切除。诊断基于组织病理学图像和免疫组化染色。只有1例患者血清IgG4水平升高。未报告复发或其他器官受累病例。
IgG4相关性疾病可能表现为孤立性胃部病变，在鉴别诊断中应予以考虑。在没有组织病理学标本检查的情况下做出最终诊断似乎很困难，可能导致误诊并随后进行不适当的治疗。IgG4-RD对类固醇治疗反应良好。然而，在这个问题上还需要进一步研究。
