Department of Nephrology, Heinrich-Heine University of Düsseldorf, Germany.
Am J Kidney Dis. 2010 Feb;55(2):259-68. doi: 10.1053/j.ajkd.2009.09.031. Epub 2009 Dec 30.
There are few anecdotal reports of circulating antineutrophil cytoplasmic autoantibodies (ANCAs) in patients with immunoglobulin A (IgA) nephropathy.
Retrospective case series.
SETTING & PARTICIPANTS: We studied 8 patients with crescentic IgA nephropathy associated with ANCAs against myeloperoxidase (n = 5) and proteinase 3 (n = 3) followed up for 2.4 +/- 1.7 years. They were compared with 26 patients with IgA nephropathy with > 10% crescentic glomeruli, but negative for ANCAs.
We analyzed clinical and histologic features of patients and their response to treatment.
Screening for ANCAs was performed using indirect immunofluorescence, and positive results were verified using enzyme-linked immunosorbent assay.
All patients with crescentic IgA nephropathy and positive for ANCAs, compared with only one-third of ANCA-negative patients, presented with the clinical syndrome of rapid progressive glomerulonephritis. ANCA-positive patients reached a higher peak serum creatinine level within the first 3 months (4.2 +/- 2.2 vs 2.5 +/- 1.9 mg/dL; estimated glomerular filtration rate, 19.3 +/- 10.2 vs 45.9 +/- 30.1 mL/min/1.73 m(2)). ANCA-positive patients with IgA nephropathy had a higher percentage of crescentic glomeruli (54.3% +/- 18%) compared with ANCA-negative patients with crescentic IgA nephropathy (34.5% +/- 26%). ANCA-positive patients were treated using cyclophosphamide and corticosteroids. Kidney function improved in all these patients: serum creatinine level decreased from the peak of 4.2 +/- 2.2 to 1.7 +/- 0.7 mg/dL at the end of follow up (estimated glomerular filtration rate, 19.3 +/- 10.2 to 44.6 +/- 11.1 mL/min/1.73 m(2)). In contrast, no significant improvement was achieved in ANCA-negative patients.
Patients with IgA nephropathy, crescents, and positive for ANCAs represent a clinical entity with a diverse more exaggerated clinical and histologic picture. However, disease in these patients responded well to aggressive immunosuppressive therapy.
有少数关于免疫球蛋白 A (IgA) 肾病患者循环抗中性粒细胞胞浆抗体 (ANCAs) 的报道。
回顾性病例系列研究。
我们研究了 8 例新月体 IgA 肾病伴髓过氧化物酶 (n = 5) 和蛋白酶 3 (n = 3) 相关 ANCAs 的患者，随访 2.4 ± 1.7 年。他们与 26 例新月体 IgA 肾病患者进行了比较，这些患者的新月体肾小球比例大于 10%，但 ANCAs 阴性。
我们分析了患者的临床和组织学特征及其对治疗的反应。
使用间接免疫荧光法进行 ANCAs 的筛查，并用酶联免疫吸附试验验证阳性结果。
与仅三分之一的 ANCAs 阴性患者相比，所有新月体 IgA 肾病伴 ANCAs 阳性的患者均表现为快速进行性肾小球肾炎的临床综合征。ANCAs 阳性患者在头 3 个月内达到更高的峰值血清肌酐水平(4.2 ± 2.2 vs 2.5 ± 1.9 mg/dL；估计肾小球滤过率，19.3 ± 10.2 vs 45.9 ± 30.1 mL/min/1.73 m(2))。与新月体 IgA 肾病的 ANCAs 阴性患者相比，IgA 肾病的 ANCAs 阳性患者的新月体肾小球比例更高(54.3% ± 18% vs 34.5% ± 26%)。ANCAs 阳性的 IgA 肾病患者接受环磷酰胺和皮质类固醇治疗。所有这些患者的肾功能均有所改善：血清肌酐水平从峰值 4.2 ± 2.2 降至随访结束时的 1.7 ± 0.7 mg/dL(估计肾小球滤过率，19.3 ± 10.2 至 44.6 ± 11.1 mL/min/1.73 m(2))。相比之下，ANCAs 阴性患者没有明显改善。
IgA 肾病、新月体和 ANCAs 阳性的患者代表一种具有更多夸张临床表现和组织学特征的临床实体。然而，这些患者的疾病对强化免疫抑制治疗反应良好。
Zotero 插件