肖慧捷 杨霁云 张焱 刘景城
摘要 目的: 加强对抗中性粒细胞胞浆抗体ANCA(antineutrophil cytoplasmic antibody, ANCA ) 相关性小血管炎(ANCA－associated small vasculitis, AASV )所致肺出血肾炎综合征的认识，探讨其发病机制、治疗和预后。方法: 根据临床特征，血清ANCA抗体阳性、抗GBM抗体阴性、肾活检病理结果，除外了Goodpasture 及其他系统性疾病。诊断为ANCA相关性小血管炎所致的肺出血肾炎综合征。并对相关文献进行复习。结果: 本病例临床表现为贫血, 肺出血、急性肾衰竭等多系统受累, 肾穿刺活检可见广泛新月体形成并有毛细血管袢纤维素样坏死, 血清学检查ANCA阳性,并除外了GBM抗体相关的肺出血肾炎综合征（Goodpasture）及系统性红斑狼疮等其它系统性疾病。但荧光检查却有免疫复合物在GBM、系膜区颗粒及短线状沉积（免疫球蛋白≥＋＋）。与经典的AASV所致肺出血肾炎综合征的免疫荧光呈阴性或弱阳性不同。结论: AASV所致的肺出血肾炎综合征确诊主要依靠ANCA 血清学检测和经皮肾穿刺活检病理,并除外其他相关的疾病。但经典的AASV所致的肺出血肾炎综合征免疫荧光呈阴性或弱阳性, 本例为国内首例儿童有免疫沉积型的以肺出血肾炎综合征为临床表现的AASV的报道。治疗主要为肾上腺皮质激素和免疫抑制剂。ANCA 试验及肾活检有利于早期诊断。早期积级治疗本病, 可有效改善预后。AASV中免疫球蛋白在肾小球阳性沉积的意义仍有待于进一步积累资料。
关键词 抗中性粒细胞胞浆抗体ANCA; ANCA相关性小血管炎; 小儿
Immune complex deposits in ANCA-associated pulmonary-renal syndrome : a case report and literature review
XIAO Huijie, YANG Jiyun, ZHANG Yan, LIU Jingcheng,
Department of Pediatric, Peking University First Hospital, Beijing, 100034
To improve the knowledge of antineutrophil cytoplasmic antibody (ANCA) associated with small vasculitis (AASV) of pulmonary-renal syndrome, with immune complex deposition within glomeruli on immunofluorescence, analyse the clinical and pathological features of ANCA associated AASV in children.
METHOD According to the clinical features, positive ANCA serology tests, negative GBM antibody, renal biopsy findings, excluded the Goodpasture's Syndrome, and other underlying systemic disease， the diagnosis was made. Related data were reviewed. compare with that of classical “pauci-immune” pulmonary-renal syndrome.
RESULT The 11-year-old girl presented with anemia, diffuse pulmonary hemorrhage in conjunction with rapidly progressive glomerulonephritis, etc. The renal biopsy displays focal segmental fibroid necrosis of the glomerular capillary tufts and diffuse crescents formation, with immune complex deposits .
CONCLUSION We report a case of ANCA-associated systemic vasculitis with immune complex deposition. AASV is a systemic disease, necrotizing and crescentic glomerulonephritis related to ANCA associated pulmonary-renal syndrome is typically referred to as “pauci-immune”. Pulmonary-renal syndrome can originate from various systemic autoimmune diseases. ANCA-associated vasculitides account for approximately 60%, Goodpasture's Syndrome for approximately 20% of the cases. Renal biopsy displays extracapillary proliferating glomerulonephritis
in which the clinical and pathological features were extracapillary proliferating glomerulonephritis, focal segmental fibroid necrosis of the glomerular capillary tufts and diffuse crescents formation, Reports of
(ANCA ) .
Intense immunosuppression with cyclophosphamide and glucocorticoids,. Early diagnosis and appropriate intensive therapy are critical for patients with idiopathic RPGN. The degree of renal function and the percent of crescents on renal biopsy are better predictors of outcome. Pediatric patients with AASV were not rare in China. The clinical and pathological features of patients with AASV in childhood were similar to adult patients .
Key words: antineutrophil cytoplasmic antibody (ANCA);
ANCA associated systemic vasculitis (AASV); child,
mainly depend on the serum of ANCA to learn the examination and