IgG4-Related Disease Is Not Associated with Antibody to the Phospholipase A2 Receptor.
IgG4-Related Disease Is Not Associated with Antibody to the Phospholipase A2 Receptor.
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DOI：
10.1155/2012/139409
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发表时间：
2012
影响因子：
2.3
通讯作者：
Stone JH
中科院分区：
文献类型：
--
作者：
Khosroshahi A;Ayalon R;Beck LH Jr;Salant DJ;Bloch DB;Stone JH
IgG 4相关疾病（IgG 4-RD）患者的组织病理学特征在受累器官中相似。在适当的临床和组织病理学背景下发现IgG 4+浆细胞浸润，将大量先前被视为单独疾病的临床实体联系起来。IgG 4-RD的肾脏受累通常以肾小管间质性肾炎为特征，但膜性肾病也被报道为IgG 4-RD的肾脏并发症之一。最近发现，高比例的特发性膜性肾病（IMN）患者在循环和肾小球免疫沉积物中具有针对M型磷脂酶A2受体（PLA 2 R）的IgG 4自身抗体，以及严重的IgG 4高丙种球蛋白血症和IgG 4-RD中膜性肾病的偶尔报告，提出了共同抗原的问题。为了评估IgG 4-RD患者中抗PLA 2 R抗体的存在，我们通过免疫印迹法筛选了28例IgG 4-RD患者的血清。该队列中没有患者具有可检测的循环抗PLA 2 R抗体。这项研究表明，尽管IgG 4-RD和IMN之间存在一些临床和血清学重叠，但抗PLA 2 R抗体在IgG 4-RD的发病机制中不起作用。有膜性肾病证据的IgG 4-RD的额外研究对于排除任何明确的关系很重要。
Patients with IgG4-related disease (IgG4-RD) share histopathological characteristics that are similar across affected organs. The finding of infiltration with IgG4+ plasma cells in the proper clinical and histopathological contexts connects a large number of clinical entities that were viewed previously as separate conditions. The renal involvement in IgG4-RD is usually characterized by tubulointerstitial nephritis, but membranous nephropathy has also been reported to be one of the renal complications of IgG4-RD. The recent discovery that a high proportion of patients with idiopathic membranous nephropathy (IMN) have IgG4 autoantibodies to the M-type phospholipase A2 receptor (PLA2R) in the circulation and glomerular immune deposits, together with the profound IgG4 hypergammaglobulinemia and occasional reports of membranous nephropathy in IgG4-RD, raised the question of a common antigen. To assess the presence of anti-PLA2R antibody in patients with IgG4-RD, we screened sera from 28 IgG4-RD patients by immunoblot. None of the patients in this cohort had detectable circulating anti-PLA2R antibodies. This study suggests that despite some clinical and serological overlaps between IgG4-RD and IMN,anti-PLA2R antibodies do not play a role in the pathogenesis of IgG4-RD. Additional studies of IgG4-RD with evidence of membranous nephropathy are important to exclude any definite relationship.