Elevated IgG4 Serum Levels Among Primary Sjogren's Syndrome Patients: Do They Unmask Underlying IgG4-Related Disease?
Elevated IgG4 Serum Levels Among Primary Sjogren's Syndrome Patients: Do They Unmask Underlying IgG4-Related Disease?
复制标题
DOI：
10.1002/acr.22216
复制
发表时间：
2014-05-01
影响因子：
4.7
通讯作者：
Moutsopoulos, Haralampos M.
中科院分区：
文献类型：
--
作者：
Mavragani, Clio P.;Fragoulis, George E.;Moutsopoulos, Haralampos M.
目的检测133例原发性干燥综合征(SS)患者和49例健康献血员的血清IgG4水平，探讨其与自身免疫性疾病的临床、血清学和组织病理学特征的关系。74例狼疮患者和54例类风湿关节炎(RA)患者作为疾病对照。结果133例原发性SS患者(高IgG4组)中有10例(7.5%)、74例狼疮患者中8例(10.8%)、54例RA患者中7例(12.9%)、49例HD患者中1例(2%)检测到升高的IgG4水平。与IgG4正常(135 mg/dl)的原发SS组相比，高IgG4患者表现出更高的IgG4相关特征(自身免疫性胆管炎、自身免疫性胰腺炎和间质性肾炎)，较低的抗核抗体阳性率，以及较高的IgG2和IgE水平。在高IgG4组的6个可用味精组织中，有3个组织检测到IgG4+浆细胞阳性染色，其IgG4：Ig G比值为40%。133例初发SS患者中有10例(7.5%)符合IgG4相关疾病(IgG4-RD)的诊断标准。结论7.5%的原发SS患者血清IgG4水平升高，且与IgG4RD相关的临床、血清学和组织病理学特征有关。这个高IgG4原发SS组是否代表一个错误分类的IgG4-RD组或一个不同的原发SS亚型仍有待于未来的研究进一步探讨。
ObjectiveTo determine IgG4 levels in a cohort of consecutive patients with primary Sjogren's syndrome (SS) and other autoimmune diseases and explore whether they associate with distinct clinical, serologic, and histopathologic features.MethodsSerum IgG4 levels were measured in 133 primary SS patients and 49 healthy donors (HDs). Seventy-four lupus and 54 rheumatoid arthritis (RA) patients served as disease controls. Immunohistochemical IgG4 analysis was performed in paraffin-embedded minor salivary gland (MSG) tissues.ResultsRaised IgG4 serum levels (> 135 mg/dl) were detected in 10 (7.5%) of 133 primary SS patients (high-IgG4 group), in 8 (10.8%) of 74 lupus patients, in 7 (12.9%) of 54 RA patients, and in 1 (2%) of 49 HDs. Compared to the normal-IgG4 (< 135 mg/dl) primary SS group, high-IgG4 patients exhibited increased prevalence of IgG4-related features (autoimmune cholangitis, autoimmune pancreatitis, and interstitial nephritis), lower rates of antinuclear antibody positivity, and higher IgG2 and IgE levels. Positive staining for IgG4+ plasma cells with an IgG4:IgG ratio >= 40% was detected in 3 of 6 available MSG tissues in the high-IgG4 group. Criteria of possible or definite IgG4-related disease (IgG4-RD) were fulfilled by 10 (7.5%) of 133 of our primary SS cohort.ConclusionRaised IgG4 serum levels were detected in 7.5% of primary SS patients in association with IgG4RD-reminiscent clinical, serologic, and histopathologic features. Whether this high-IgG4 primary SS group represents a misclassified IgG4-RD group or a distinct primary SS subtype remains to be further explored in future studies.