Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA 95616, USA.
Autoimmun Rev. 2012 May;11(6-7):A404-12. doi: 10.1016/j.autrev.2011.12.001. Epub 2011 Dec 8.
The role of specific immunoglobulin isotypes in human autoimmune disease has long attracted attention. Indeed, the presence of a polyclonal gammopathy is well known in a variety of systemic autoimmune diseases and is likely the result of chronic inflammation. However, in specific clinical situations, patients manifest isolated and elevated IgM levels, but normal IgG and IgA. The pathophysiology of this elevation and the clinical significance have been elusive. However, the relationships between specific genes and hyper-IgM are now very well defined, as it has been documented in primary hyper IgM syndromes. In this review we present data on clinical diseases with characteristic IgM abnormalities, including primary and secondary hyper IgM syndromes, autoimmune hemolytic anemia, cryoglobulinemia, primary biliary cirrhosis and multiple sclerosis and place the data in the perspective of the normal maturation of the immune response, including somatic mutation and genetic rearrangement.
特定免疫球蛋白亚型在人类自身免疫性疾病中的作用一直备受关注。事实上，多种系统性自身免疫性疾病中存在多克隆丙种球蛋白血症，这很可能是慢性炎症的结果。然而，在特定的临床情况下，患者表现出孤立性和升高的 IgM 水平，但 IgG 和 IgA 正常。这种升高的病理生理学及其临床意义一直难以捉摸。然而，现在已经非常明确特定基因与高 IgM 之间的关系，因为已经在原发性高 IgM 综合征中得到了证实。在这篇综述中，我们介绍了具有特征性 IgM 异常的临床疾病的数据，包括原发性和继发性高 IgM 综合征、自身免疫性溶血性贫血、冷球蛋白血症、原发性胆汁性肝硬化和多发性硬化症，并将这些数据置于正常免疫反应成熟的背景下，包括体细胞突变和基因重排。
