Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Department of Pathology and Laboratory Medicine, King Abdullah University Hospital, Irbid, Jordan.
Am J Case Rep. 2020 Oct 27;21:e926803. doi: 10.12659/AJCR.926803.
BACKGROUND Immunoglobulin G4 (IgG4)-related disease is immune-mediated and was first proposed as a defined entity after studies on patients with autoimmune pancreatitis. Since then, it has been reported in many organs. Involvement of the ovaries is rare, and to our knowledge, only 2 cases have been reported in the literature. IgG4-related disease is associated with increased serum IgG4 levels. Organ involvement includes a lymphoplasmacytic infiltrate, fibrosis, and obliterative phlebitis, with immunohistochemistry showing IgG4-positive plasma cells. This report is of a case of IgG4-related disease involving the right ovary. CASE REPORT A 47-year-old woman presented with a right ovarian cyst. An ultrasound scan revealed a complex right ovarian cyst with multiple septations. The hormonal profile and tumor markers were unremarkable. Gross examination showed fragments of cyst wall. Histologic examination revealed a follicular cyst, surrounded by a dense lymphoplasmacytic infiltrate rich in eosinophils, partially obliterative phlebitis, and fibrosis. Immunohistochemically, IgG marked most of the plasma cells, of which 70% expressed IgG4, with a count >50 cells per high-power field. Subsequent testing of serum IgG4 showed that the level was elevated (330 mg/dL). A diagnosis of IgG4-related disease was made. CONCLUSIONS Ovarian involvement by IgG4-related disease is rarely described in the literature. Our patient is likely to be the third case. We believe that cumulative findings from our case along with the 2 already reported cases increase awareness and may establish a framework for building more objective criteria to define this entity in the ovaries, similar to what has been achieved in some other organs.
免疫球蛋白 G4（IgG4）相关疾病是一种免疫介导的疾病，最初是在研究自身免疫性胰腺炎患者后作为一种明确的疾病提出的。此后，它已在许多器官中报道。卵巢受累很少见，据我们所知，文献中仅报道了 2 例。IgG4 相关疾病与血清 IgG4 水平升高有关。器官受累包括淋巴浆细胞浸润、纤维化和闭塞性静脉炎，免疫组化显示 IgG4 阳性浆细胞。本报告介绍了一例 IgG4 相关疾病累及右侧卵巢。
一名 47 岁女性因右侧卵巢囊肿就诊。超声检查显示右侧卵巢有一个复杂的囊肿，有多个分隔。激素谱和肿瘤标志物无异常。大体检查显示囊壁碎片。组织学检查显示滤泡囊肿，周围有丰富嗜酸性粒细胞的致密淋巴浆细胞浸润，部分闭塞性静脉炎和纤维化。免疫组化检查显示 IgG 标记大多数浆细胞，其中 70%表达 IgG4，每高倍视野计数＞50 个细胞。随后检测血清 IgG4 水平升高（330mg/dL）。诊断为 IgG4 相关疾病。
IgG4 相关疾病累及卵巢在文献中很少见。我们的患者可能是第三例。我们认为，我们的病例以及已经报道的 2 例病例的累积结果提高了认识，并可能为在卵巢中建立更客观的定义该实体的标准奠定基础，类似于在一些其他器官中已经实现的标准。
