Bateman Adrian C, Ashton-Key Margaret R, Jogai Sanjay
Department of Cellular Pathology, University Hospital Southampton NHS Foundation Trust, Southampton, UK.
Histopathology. 2015 Oct;67(4):557-61. doi: 10.1111/his.12658. Epub 2015 Mar 5.
Immunoglobulin (Ig)G4-related disease (IgG4-RD) is an increasingly recognized fibroinflammatory condition that commonly exhibits multisystem involvement, with localized (e.g. inflammatory pseudotumours that can mimic malignancy) or diffuse (leading to organ dysfunction) patterns of tissue involvement. The 2012 Boston criteria have standardized the histopathological approach to the diagnosis of IgG4-RD and require one or more of the cardinal morphological features with prominence of IgG4(+) plasma cells and an IgG4(+) /IgG(+) plasma cell ratio of at least 40%. The relative prevalence of the morphological criteria varies between anatomical sites, but granulomas are rarely found and, indeed, their presence would usually deter a pathologist from making this diagnosis. The aim was to characterize two cases of IgG4-RD in which granulomas were present and to highlight this as an unusual feature of the condition.
We describe two cases in which the features of IgG4-RD were present within lymph nodes, together with granulomas. This is a recognized but rare morphological pattern of IgG4-RD.
While an unusual finding, the presence of granulomas should not preclude a diagnosis of IgG4-RD in the appropriate clinicopathological context.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种日益受到认可的纤维炎性疾病，通常表现为多系统受累，有局部（如可酷似恶性肿瘤的炎性假瘤）或弥漫性（导致器官功能障碍）的组织受累模式。2012年波士顿标准对IgG4-RD的组织病理学诊断方法进行了标准化，要求具备一项或多项主要形态学特征，即IgG4(+)浆细胞显著增多且IgG4(+) /IgG(+)浆细胞比例至少为40%。形态学标准的相对患病率在不同解剖部位有所不同，但肉芽肿很少见，实际上，肉芽肿的存在通常会使病理学家难以做出该诊断。目的是对两例存在肉芽肿的IgG4-RD病例进行特征描述，并强调这是该疾病的一个不寻常特征。
我们描述了两例病例，其中淋巴结内存在IgG4-RD的特征以及肉芽肿。这是一种已被认可但罕见的IgG4-RD形态学模式。
虽然肉芽肿的存在是一个不寻常的发现，但在适当的临床病理背景下，不应排除IgG4-RD的诊断。
