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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 clinicopathological features, diagnosis and prognosis of ANCA-associated nephritis with IgA nephropathy. Methods The clinical data of 3 patients with ANCA-associated glomerulonephritis and IgA nephropathy were retrospectively analyzed. The pathological changes and immunohistochemistry were observed by light microscopy, and the related literatures were reviewed. Results 2 cases of young women, 1 case of middle-aged men. 1 case of MPO-ANCA (+), 1 case of PR3-ANCA (+), 1 case of two antibodies (+). Renal damage occurred in 3 cases, of which case 2 showed symptoms of gastrointestinal tract such as acid reflux and diarrhea during follow-up. Example 3 had atelectasis, pleural effusion and pericardial effusion. Three cases of immunohistochemical staining showed that Ig A was deposited granularly in the mesangial area. Three patients were given hormone combined with cyclophosphamide, 1 patient completely relieved, 2 patients partially relieved. CONCLUSIONS: ANCA-associated nephritis is still rare in IgA nephropathy. The exact pathogenesis is unknown. Another diagnosis is easily overlooked when light microscopy shows a pathological feature. Clear diagnosis should be combined with histopathology, serology and immunohistochemistry.