Al-Khalili Orwah M, Erickson Alan R
Orwah M. Al-Khalili, MD, is a Rheumatology Fellow, University of Nebraska Medical Center, Omaha, Neb.
Alan R. Erickson, MD, is an Associate Professor of Internal Medicine and Rheumatology, University of Nebraska Medical Center, Omaha, Neb.
Mo Med. 2018 May-Jun;115(3):253-256.
IgG4-related disease (IgG-RD) describes a group of fibroinflammatory diseases that affect a variety of tissues resulting in tumor-like effect and/or organ dysfunction. Clinical presentation varies according to the tissue(s) involved, and diagnosis relies on tissue findings of dense infiltration of IgG4-positive plasma cells and a characteristic storiform fibrosis. Treatment is mainly with glucocorticoids, while multiple immunosuppressive medications can be used as adjuvant agents. Rituximab has showed promising results, but further studies are needed.
IgG4相关疾病（IgG-RD）是指一组纤维炎性疾病，可累及多种组织，导致肿瘤样效应和/或器官功能障碍。临床表现因受累组织而异，诊断依赖于IgG4阳性浆细胞密集浸润和特征性席纹状纤维化的组织学表现。治疗主要使用糖皮质激素，同时多种免疫抑制药物可作为辅助药物。利妥昔单抗已显示出有前景的结果，但仍需进一步研究。
