Bhardwaj S, Goyal S, Yadav A K, Goyal A
Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India.
J Postgrad Med. 2018 Apr-Jun;64(2):119-122. doi: 10.4103/jpgm.JPGM_778_16.
IgG4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease entity, commonly creating confusion and diagnostic challenges. We present a case of a 25-year-old female who presented with bilateral orbital masses, lymphadenopathy, paraspinal and renal masses, which clinicoradiologically simulated lymphoma. The lymph node biopsy revealed interfollicular sheets of plasma cells creating confusion with Castleman's disease and marginal zone lymphoma. The orbital biopsy revealed ductular destruction, periductular plasma cells, and fibrosis, mimicking Sjogren's syndrome and Castleman's disease. However, the correlation of the clinical features with histopathological findings, IgG4 immunopositivity, and serum studies helped in clinching the diagnosis. This case presents an uncommon combination of clinical features infrequently reported in literature. Furthermore, and more importantly, it highlights the need to keep a differential of IgG4-RD in mind, to aid early and correct treatment of the disease.
IgG4相关性疾病（IgG4-RD）是一种多系统的形成肿块的免疫介导性疾病实体，常造成诊断上的混淆和挑战。我们报告一例25岁女性病例，该患者出现双侧眼眶肿块、淋巴结病、椎旁和肾脏肿块，临床放射学表现疑似淋巴瘤。淋巴结活检显示滤泡间浆细胞成片，这与Castleman病和边缘区淋巴瘤相混淆。眼眶活检显示导管破坏、导管周围浆细胞浸润和纤维化，类似干燥综合征和Castleman病。然而，临床特征与组织病理学结果、IgG4免疫阳性及血清学检查结果之间的相关性有助于明确诊断。该病例呈现出文献中罕见报道的不常见临床特征组合。此外，更重要的是，它强调了需要考虑到IgG4-RD的鉴别诊断，以帮助早期正确治疗该疾病。
