Department of Pediatrics, Korea University Medical Center, Seoul, Korea.
J Korean Med Sci. 2012 Nov;27(11):1436-8. doi: 10.3346/jkms.2012.27.11.1436. Epub 2012 Oct 30.
Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.
各种形式的低丙种球蛋白血症可发生在自身免疫性疾病患者中,反之亦然。我们报告了一例 13 岁男孩,患有膜性肾病和普通可变免疫缺陷。他表现为肾病综合征、肺炎伴支气管扩张和严重的低丙种球蛋白血症。肾活检显示弥漫性增厚的肾小球毛细血管壁有“尖峰”,提示膜性肾病。通过实验室研究排除了继发性病因;然而,类固醇治疗后蛋白尿仍持续存在。加用环孢素和静脉注射免疫球蛋白后,患者出院时蛋白尿减少。低丙种球蛋白血症可能对某些膜性肾病患者的免疫失调产生有害影响。