Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders
Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders
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DOI：
10.1136/ard.2008.089169
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发表时间：
2009-08-01
影响因子：
27.4
通讯作者：
Umehara, H.
中科院分区：
文献类型：
--
作者：
Masaki, Y.;Dong, L.;Umehara, H.
背景：Mikulicz病（MD）被认为是干燥综合征（SS）的一种表现。目的：探讨IgG（4）相关疾病（包括MD和SS）之间的差异。方法：对在日本登记的MD和IgG（4）相关疾病患者进行研究，并建立新的临床实体IgG（4）阳性多器官淋巴组织增生综合征（IgG（4）+MOLPS）的临时标准。初步诊断标准包括血清IgG（4）水平升高（>135 mg/dl）和组织中IgG（4）（+）浆细胞浸润（IgG（4）+/IgG+浆细胞>50%）伴纤维化或硬化。结果：IgG（4）+MOLPS组口干、干眼症、关节痛、类风湿因子、抗核抗体、抗SS-A/Ro、抗SS-B/La抗体的发生率明显低于典型SS组（P <0.05）。在IgG（4）+MOLPS组中，变应性鼻炎和自身免疫性胰腺炎的发生率显著更高，总IgG、IgG（2）、IgG（4）和IgE水平显著升高。IgG（4）+MOLPS患者的组织学标本显示明显的IgG（4）+浆细胞浸润。许多IgG（4）+MOLPS患者有淋巴细胞滤泡形成，但淋巴上皮病变罕见。典型SS患者组织中可见少量IgG（4）+细胞。38例IgG（4）+MOLPS患者经糖皮质激素治疗后，临床症状明显改善。结论：IgG（4）+MOLPS与SS虽然受累器官相似，但在临床和病理上有很大差异。根据其临床特点和对糖皮质激素的良好反应，我们提出了一个新的临床实体：IgG（4）+MOLPS。
Background: Mikulicz's disease (MD) has been considered as one manifestation of Sjogren's syndrome (SS). Recently, it has also been considered as an IgG(4)-related disorder.Objective: To determine the differences between IgG(4)-related disorders including MD and SS.Methods: A study was undertaken to investigate patients with MD and IgG(4)-related disorders registered in Japan and to set up provisional criteria for the new clinical entity IgG(4)-positive multiorgan lymphoproliferative syndrome (IgG(4)+MOLPS). The preliminary diagnostic criteria include raised serum levels of IgG(4) (>135 mg/dl) and infiltration of IgG(4)(+) plasma cells in the tissue (IgG(4)+/IgG+ plasma cells >50%) with fibrosis or sclerosis. The clinical features, laboratory data and pathologies of 64 patients with IgG(4)+MOLPS and 31 patients with typical SS were compared.Results: The incidence of xerostomia, xerophthalmia and arthralgia, rheumatoid factor and antinuclear, antiSS-A/Ro and antiSS-B/La antibodies was significantly lower in patients with IgG(4)+MOLPS than in those with typical SS. Allergic rhinitis and autoimmune pancreatitis were significantly more frequent and total IgG, IgG(2), IgG(4) and IgE levels were significantly increased in IgG(4)+MOLPS. Histological specimens from patients with IgG(4)+MOLPS revealed marked IgG(4)+ plasma cell infiltration. Many patients with IgG(4)+MOLPS had lymphocytic follicle formation, but lymphoepithelial lesions were rare. Few IgG(4)+ cells were seen in the tissue of patients with typical SS. Thirty-eight patients with IgG(4)+MOLPS treated with glucocorticoids showed marked clinical improvement.Conclusion: Despite similarities in the involved organs, there are considerable clinical and pathological differences between IgG(4)+MOLPS and SS. Based on the clinical features and good response to glucocorticoids, we propose a new clinical entity: IgG(4)+MOLPS.