Berezovskiy Reanay, Crawford James M, Rishi Arvind, Khurana Sohil, Kern Joshua, Morscher Stefani, Satapathy Sanjaya K
New York Institute of Technology College of Osteopathic Medicine, Old Westbury, NY.
Northwell Health, Hempstead, NY.
ACG Case Rep J. 2024 Sep 20;11(9):e01481. doi: 10.14309/crj.0000000000001481. eCollection 2024 Sep.
IgG4-related disease (IgG4-RD) is an autoimmune syndrome that is characterized by elevated levels of serum IgG4 and infiltration of various tissue types by IgG4 immunoreactive plasma cells. The IgG4-RD can result in systemic disease and the formation of inflammatory mass lesions, frequently addressed as pseudotumors. While IgG4-RD can manifest in various organs, liver involvement is rare, and because it is an immune-mediated inflammatory process, it is uncommon in patients who are immunocompromised. Furthermore, despite IgG4-RD responding well to immunosuppressive treatment, cases of spontaneous remission are exceedingly rare in the literature. In this report, we present the unique case of a self-resolving IgG4-RD lesion of the liver in a HIV positive patient.
IgG4相关性疾病(IgG4-RD)是一种自身免疫综合征,其特征为血清IgG4水平升高以及IgG4免疫反应性浆细胞浸润多种组织类型。IgG4-RD可导致全身性疾病并形成炎性肿块病变,常被称为假瘤。虽然IgG4-RD可在多个器官表现出来,但肝脏受累情况罕见,并且由于它是一种免疫介导的炎症过程,在免疫功能低下的患者中并不常见。此外,尽管IgG4-RD对免疫抑制治疗反应良好,但文献中自发缓解的病例极为罕见。在本报告中,我们呈现了一例HIV阳性患者肝脏IgG4-RD病变自行消退的独特病例。