Sodavarapu Soujanya, Ghotra Gurinder S, Obad Nashwan, Goyal Megha, Gill Amandeep S
Internal Medicine, San Joaquin General Hospital, French Camp, USA.
Internal Medicine, San Jaoquin General Hospital, French Camp, USA.
Cureus. 2020 Jan 9;12(1):e6610. doi: 10.7759/cureus.6610.
Immunoglobulin G4-related disease (IgG4-RD) is a chronic multisystem immune-mediated disease. Histologically, it presents as infiltration with IgG4-secreting plasma cells affecting many organs such as the pancreas, lacrimal glands, salivary glands, kidneys, and arteries, resulting in chronic fibrosis and scarring within the tissue which over time forms into a mass. It is a slow-growing process that usually remains indolent until it causes a mass effect or tissue infiltration. Our patient was found to have a pancreatic mass on imaging concerning pancreatic neoplastic lesions. He subsequently underwent biopsy for histology/pathology and IgG4 levels, which led to our diagnosis of IgG4-RD. Imaging, blood tests, and, most importantly, histopathological features of the involved organ are important in determining the diagnosis. This is important as a treatment plan will be based on the given diagnosis, and patients with IgG4-RD respond very well to systemic steroid therapy.
免疫球蛋白G4相关性疾病(IgG4-RD)是一种慢性多系统免疫介导性疾病。在组织学上,它表现为分泌IgG4的浆细胞浸润,可累及许多器官,如胰腺、泪腺、唾液腺、肾脏和动脉,导致组织内慢性纤维化和瘢痕形成,随着时间的推移形成肿块。这是一个生长缓慢的过程,通常在引起占位效应或组织浸润之前处于惰性状态。我们的患者在影像学检查中发现胰腺有肿块,怀疑为胰腺肿瘤性病变。随后他接受了活检以进行组织学/病理学检查及检测IgG4水平,最终确诊为IgG4-RD。影像学检查、血液检查,以及最重要的是受累器官的组织病理学特征,对于确定诊断至关重要。这一点很重要,因为治疗方案将基于给定的诊断,而IgG4-RD患者对全身类固醇治疗反应良好。