Comparison of clinical and pathological features of lung lesions of systemic IgG4-related disease and idiopathic multicentric Castleman's disease
Comparison of clinical and pathological features of lung lesions of systemic IgG4-related disease and idiopathic multicentric Castleman's disease
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DOI：
10.1111/his.13186
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发表时间：
2017-06-01
期刊：
影响因子：
6.4
通讯作者：
Ogura, Takashi
中科院分区：
文献类型：
--
作者：
Terasaki, Yasuhiro;Ikushima, Soichiro;Ogura, Takashi
目的IgG 4相关疾病（IgG 4-RD）的肺部病变[免疫球蛋白（IG）G4-L]是与IgG 4-RD同时发生的一种疾病，通常与特发性多中心Castleman病（iMCD）的肺部病变[特发性多中心Castleman病（iMCD-L）]相似。因为没有临床和病理研究以前比较这些疾病的特点，我们进行了比较与临床和组织学data.Methods和resultsNine患者有IgG 4-L（高水平的血清IgG 4和IgG 4（+）细胞在肺标本，典型的肺外表现）。15例患者患有iMCD-L（多克隆高免疫球蛋白血症、血清白细胞介素-6水平升高和多发性淋巴结病伴典型淋巴结病性病变）。IgG 4-L组的年龄、血清血红蛋白水平和IgG 4/IgG比值的平均值较高，iMCD-L组的C反应蛋白水平较高。所有IgG 4-RD肺病变均显示粘液瘤性肉芽肿样纤维化（活动性纤维化），淋巴周围基质区域（如小叶间隔和胸膜）内有淋巴浆细胞浸润和散在嗜酸性粒细胞，伴阻塞性血管炎。然而，所有15个肺病变的iMCD，有明显的多克隆淋巴浆细胞的积累与淋巴滤泡和致密的纤维化病变，主要是在肺泡区相邻的小叶间隔和胸膜没有阻塞性vasculitis.ConclusionsAlthough这两个病变有淋巴浆细胞浸润，肺病变的IgG 4-RD的特点是积极的纤维化与嗜酸性粒细胞浸润的周围淋巴基质区与阻塞性vasculitis. ConclusionsAlthough，而iMCD的肺部病变主要在邻近外淋巴基质区的肺泡区存在淋巴浆细胞增生病变。这些临床病理特征可能有助于鉴别这两种疾病。
AimsThe lung lesion [immunoglobulin (Ig)G4-L] of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion [idiopathic multicentric Castleman's disease (iMCD-L)] of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data.Methods and resultsNine patients had IgG4-L (high levels of serum IgG4 and of IgG4(+) cells in lung specimens; typical extrapulmonary manifestations). Fifteen patients had iMCD-L (polyclonal hyperimmunoglobulinaemia, elevated serum interleukin-6 levels and polylymphadenopathy with typical lymphadenopathic lesions). Mean values for age, serum haemoglobin levels and IgG4/IgG ratios were higher in the IgG4-L group and C-reactive protein levels were higher in the iMCD-L group. All IgG4-RD lung lesions showed myxomatous granulation-like fibrosis (active fibrosis), with infiltration of lymphoplasmacytes and scattered eosinophils within the perilymphatic stromal area, such as interlobular septa and pleura with obstructive vasculitis. All 15 lung lesions of iMCD, however, had marked accumulation of polyclonal lymphoplasmacytes in lesions with lymphoid follicles and dense fibrosis, mainly in the alveolar area adjacent to interlobular septa and pleura without obstructive vasculitis.ConclusionsAlthough both lesions had lymphoplasmacytic infiltration, lung lesions of IgG4-RD were characterized by active fibrosis with eosinophilic infiltration within the perilymphatic stromal area with obstructive vasculitis, whereas lung lesions of iMCD had lymphoplasmacyte proliferating lesions mainly in the alveolar area adjacent to the perilymphatic stromal area. These clinicopathological features may help to differentiate the two diseases.