ANCA相关性肾炎合并IgA肾病3例临床病理分析

ANCA-associated glomerulonephritis combined with IgA nephropathy:a clinicopathological analysis of thress cases

目的探讨ANCA相关性肾炎合并Ig A肾病的临床病理学特征、诊断及预后。方法回顾性分析3例ANCA相关性肾炎合并Ig A肾病肾穿组织的临床资料、光镜病理改变、免疫组化结果,并复习相关文献。结果 2例为年轻女性,1例为中年男性。1例MPO-ANCA(+),1例PR3-ANCA(+),1例2种抗体均(+)。3例均出现肾损害,其中例2在随访时出现反酸、腹泻等胃肠道累及症状,例3伴肺不张、胸腔积液及心包积液。3例免疫组化染色显示Ig A在肾小球系膜区颗粒状沉积。3例均给予激素联合环磷酰胺治疗,1例完全缓解,2例部分缓解。结论ANCA相关性肾炎合并Ig A肾病仍非常罕见,具体发病机制不明,当光镜下突出表现出一种疾病病理特征时,容易忽略另一诊断。明确诊断需结合组织病理学、血清学及免疫组织化学。

Objective To investigate the clinical and pathological characteristics,diagnosis and prognosis of ANCAassociated glomerulonephritis combined with Ig A nephropathy.Methods The clinical data,pathological changes and immunohistochemical staining of three cases of ANCA-GN with Ig A N were analyzed retrospectively with review of the related literatures and follow-up.Results Two cases were young women and one case was male;MPOand ANCA were positive in two cases;PR3-ANCA was observed in one case.All appeared renal damage,and follow-up study showed that the second case had gastrointestinal involvement and presented with acid reflux,diarrhea,and the third presented with atelectasis,pleural effusion and pericardial effusion.All showed relatively strong deposits of Ig A in mesangial area.One achieved complete remission and other partial remission after being treated with hormone combined with cyclophosphamide and glucocorticoids;the second and third showed chronic renal failure lately.Conclusions The coexistence of ANCAGN and Ig AN is rare.However,the pathogenesis remains unclear.It is easy to neglect another diagnosis when the disease presents only one pathological feature under the light microscope.Definite diagnosis depends on its histopathology,serology and immunohistochemistry.