Tamura A, Agematsu K, Urasawa R, Naganuma K, Komiyama A
Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
Eur J Pediatr. 1998 Jun;157(6):475-8. doi: 10.1007/s004310050857.
Systemic lupus erythematosus (SLE) was observed in a 7-year-old boy with IgG2 and IgG4 subclass deficiencies who had been treated with immunoglobulin (100-200 mg/kg/4 weeks) since 2 years of age. The mother and the half-brother displayed the same deficiency. Serum IgG mainly consisted of IgG1 (98.9%) during the acute phase of SLE due to transient IgG3 deficiency. While he had no common manifestations of SLE such as arthritis or nephropathy, he developed cardiac tamponade due to massive pericardial effusion.
The clinical features of SLE in the present case such as the development of cardiac tamponade and the absence of renal involvement may result from the markedly imbalanced IgG subclass distribution among auto-antibodies.
一名7岁男孩患有IgG2和IgG4亚类缺陷,自2岁起接受免疫球蛋白治疗(100 - 200 mg/kg/4周),观察到其患有系统性红斑狼疮(SLE)。母亲和同父异母的兄弟表现出相同的缺陷。在SLE急性期,由于短暂的IgG3缺陷,血清IgG主要由IgG1组成(98.9%)。虽然他没有SLE的常见表现,如关节炎或肾病,但因大量心包积液发展为心脏压塞。
本病例中SLE的临床特征,如心脏压塞的发生和无肾脏受累,可能是由于自身抗体中IgG亚类分布明显失衡所致。