Greenbaum Alissa, Yadak Nour, Perez Steven, Rajput Ashwani
Department of Surgery, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA.
Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA.
BMJ Case Rep. 2017 Jun 8;2017:bcr-2017-220400. doi: 10.1136/bcr-2017-220400.
IgG-related disease (IgG-RD) is a rare form of autoimmune sclerosing disease, characterised by elevated serum IgG and tissue IgG levels, specific histopathological findings, multiorgan involvement and adequate response to glucocorticoid treatment. The low incidence and the heterogeneous nature of the disease has made consensus on diagnostic criteria for IgG-RD difficult. Whether sclerosing mesenteritis (SM) is considered a manifestation of IgG-RD is strongly debated. We present the case of a patient with a history of rheumatoid arthritis who presented with a calcified abdominal mass. She was found to have an isolated, pedunculated mesenteric mass positive for IgG and concurrently elevated serum IgG levels. Clinical features did not classify her disease as either SM or IgG-RD as currently described in consensus statements. Concurrent diagnoses of IgG-RD, SM and other autoimmune disorders, as well as postoperative recommendations for resected isolated IgG-positive masses, are discussed.
IgG相关疾病（IgG-RD）是一种罕见的自身免疫性硬化性疾病，其特征为血清IgG和组织IgG水平升高、特定的组织病理学表现、多器官受累以及对糖皮质激素治疗有充分反应。该疾病的低发病率和异质性使得就IgG-RD的诊断标准达成共识变得困难。硬化性肠系膜炎（SM）是否被视为IgG-RD的一种表现存在激烈争论。我们报告一例有类风湿关节炎病史的患者，该患者出现腹部钙化肿块。她被发现有一个孤立的、带蒂的肠系膜肿块，IgG检测呈阳性，同时血清IgG水平升高。根据目前共识声明中的描述，其临床特征既不能将她的疾病归类为SM，也不能归类为IgG-RD。本文讨论了IgG-RD、SM和其他自身免疫性疾病的并发诊断，以及对切除的孤立IgG阳性肿块的术后建议。
