抗肾小球基底膜肾炎合并抗中性粒细胞胞质抗体相关性血管炎和IgA肾病1例  

A case of anti-glomerular basement membrane nephritis complicated with anti-neutrophil cytoplasmic antibody associated vasculitis and IgA nephropathy

作　　者：罗丽花 杨榆娟 张治平 马继孔 张华英 Luo Lihua;Yang Yujuan;Zhang Zhiping;Ma Jikong;Zhang Huaying(Department of Nephrology,Dali Bai Autonomous Prefecture People's Hospital,Dali Bai Autonomous Prefecture 671000,China)

机构地区：[1]大理白族自治州人民医院肾内科,大理白族自治州671000

出　　处：《中华肾脏病杂志》2024年第5期396-399,共4页Chinese Journal of Nephrology

摘　　要：抗肾小球基底膜(glomerular basement membrane,GBM)肾炎和抗中性粒细胞胞质抗体(anti⁃neutrophil cytoplasmic antibody,ANCA)相关性血管炎是均可累及全身多系统的自身免疫性疾病,均能引起新月体性肾小球肾炎。目前国内外对这类“双阳性”抗体的病例均有报道,但抗GBM和ANCA同时阳性的急进性肾小球肾炎(acute progressive glomerulonephritis,RPGN)合并IgA肾病的病例较为罕见。本病例为中年女性患者,其临床表现符合RPGN,抗GBM抗体及抗髓过氧化物酶阳性,经肾穿刺活检诊断为抗GBM肾炎合并ANCA相关性血管炎和IgA肾病。经血浆置换、血液透析、糖皮质激素、环磷酰胺等治疗后,病情好转出院。但出院后患者未遵医嘱服药及继续血液透析治疗,病情加重,最终死亡。Anti-glomerular basement membrane(GBM)nephritis and anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis are both autoimmune diseases that can involve multiple systems throughout the body,and both can cause crescent glomerulonephritis.At present,cases of such"double-positive"antibodies have been reported at home and abroad,but the cases of acute progressive glomerulonephritis(RPGN)with IgA nephropathy that are both positive for GBM and ANCA are relatively rare.This case was a middle-aged female patient,whose clinical manifestations were consistent with rapidly progressive glomerulonephritis,positive for anti-GBM antibodies and anti-myeloperoxidase.After renal puncture biopsy,she was diagnosed as anti-GBM nephritis complicated with ANCA associated vasculitis and IgA nephropathy.After treatment with plasma exchange,hemodialysis,glucocorticoids,cyclophosphamide,etc.,the condition improved and the patient was discharged.But the patient did not follow the doctor's instructions to take medication and continue hemodialysis treatment after discharge,and the condition worsened,ultimately leading to death.

关 键 词：抗肾小球基膜疾病 抗体 抗中性白细胞胞质 肾小球肾炎 IgA 急进性肾小球肾炎 

分 类 号：R692.31[医药卫生—泌尿科学]