Weindorf Steven C, Frederiksen John Karl
From the Department of Pathology, University of Michigan, Ann Arbor. Dr Frederiksen is now with the Department of Pathology, Jackson Memorial Hospital, Miami, Florida.
Arch Pathol Lab Med. 2017 Nov;141(11):1476-1483. doi: 10.5858/arpa.2017-0257-RA.
IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4 plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy. Pathologists thus play a key role in the diagnosis of IgG4-RD, and familiarity with its histopathologic features is essential to preventing the irreversible comorbidities associated with this treatable disease. This brief review outlines the epidemiology, clinical manifestations, and histopathology of IgG4-RD, with the aim of furthering pathologists' awareness of and ability to diagnose this disorder.
IgG4相关疾病（IgG4-RD）是一种全身性自身免疫性纤维炎性疾病，可产生硬化性、肿块样病变，其中含有富含免疫球蛋白（Ig）G4浆细胞的致密淋巴浆细胞浸润。IgG4-RD最初被描述为自身免疫性胰腺炎的一种形式，现在几乎在每个器官系统中都发现了其独特的组织病理学特征。然而，由于IgG4-RD的临床表现多样且不具特异性，在获取活检或切除标本以诊断疑似恶性肿瘤之前，该疾病可能未被怀疑。因此，病理学家在IgG4-RD的诊断中起着关键作用，熟悉其组织病理学特征对于预防与这种可治疗疾病相关的不可逆合并症至关重要。本简要综述概述了IgG4-RD的流行病学、临床表现和组织病理学，旨在提高病理学家对该疾病的认识以及诊断能力。
