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Abstract：
BACKGROUND: Immune-mediated vasculitis with 2 or more autoantibodies, for example, anti-proteinase-3, combined with anti-myeloperoxidase (MPO) or anti-glomerular basement membrane (GBM) antibodies, is extremely unusual. Furthermore, the coexistence of autoimmune vasculitis and hematological malignancies is uncommon. Herein, we describe a case of double-seropositive anti-neutrophil cytoplasmic antibody (ANCA) vasculitis with multiple myeloma.
METHODS: A 79-year-old Asian man presented with persistent leg edema and kidney dysfunction. His kidney function rapidly decreased, and serologic test results showed higher titers of the anti-MPO antibody (54.7 IU/mL) and anti-GBM antibodies (>200 IU/mL). Additionally, the clinical features showed the possibility of monoclonal gammopathy with anemia and hyperglobulinemia. We performed kidney and bone marrow biopsy. Serum protein electrophoresis and immunofixation revealed no significant differences, but the results of the bone marrow smear were compatible with those of myeloma with 15% plasmacytosis. However, kidney biopsy showed diffuse crescentic glomerulonephritis without deposition of the immune complex or kappa/lambda chain.
METHODS: Finally, the patient was diagnosed with double-seropositive ANCA-associated glomerulonephritis and multiple myeloma. Given the patient\'s performance status, we initiated low-dose steroid pulse therapy, followed by conservative management.
RESULTS: While the pulmonary lesions showed improvement, the kidney function did not regain its previous state, prompting the initiation of kidney replacement therapy by hemodialysis. There has been a decrease in the levels of anti-GBM and anti-MPO antibodies since the initial diagnosis.
CONCLUSIONS: This case elucidates the complex interplay between ANCA-associated glomerulonephritis and hematologic malignancy and emphasizes the need for a nuanced treatment strategy considering its multifaceted clinical presentation.
METHODS: A 79-year-old Asian man presented with persistent leg edema and kidney dysfunction. His kidney function rapidly decreased, and serologic test results showed higher titers of the anti-MPO antibody (54.7 IU/mL) and anti-GBM antibodies (>200 IU/mL). Additionally, the clinical features showed the possibility of monoclonal gammopathy with anemia and hyperglobulinemia. We performed kidney and bone marrow biopsy. Serum protein electrophoresis and immunofixation revealed no significant differences, but the results of the bone marrow smear were compatible with those of myeloma with 15% plasmacytosis. However, kidney biopsy showed diffuse crescentic glomerulonephritis without deposition of the immune complex or kappa/lambda chain.
METHODS: Finally, the patient was diagnosed with double-seropositive ANCA-associated glomerulonephritis and multiple myeloma. Given the patient\'s performance status, we initiated low-dose steroid pulse therapy, followed by conservative management.
RESULTS: While the pulmonary lesions showed improvement, the kidney function did not regain its previous state, prompting the initiation of kidney replacement therapy by hemodialysis. There has been a decrease in the levels of anti-GBM and anti-MPO antibodies since the initial diagnosis.
CONCLUSIONS: This case elucidates the complex interplay between ANCA-associated glomerulonephritis and hematologic malignancy and emphasizes the need for a nuanced treatment strategy considering its multifaceted clinical presentation.
摘要：
背景：具有2种或更多种自身抗体的免疫介导性血管炎，例如,抗蛋白酶-3，结合抗髓过氧化物酶（MPO）或抗肾小球基底膜（GBM）抗体，是非常不寻常的。此外，自身免疫性血管炎和恶性血液病并存并不常见.在这里，我们描述了1例双血清阳性抗中性粒细胞胞浆抗体(ANCA)血管炎伴多发性骨髓瘤的病例.
方法：一名79岁的亚裔男子表现为持续性腿部水肿和肾功能不全。他的肾功能迅速下降，血清学检测结果显示抗MPO抗体(54.7IU/mL)和抗GBM抗体(>200IU/mL)的滴度较高.此外,临床特征显示单克隆丙种球蛋白病合并贫血和高球蛋白血症的可能性。我们进行了肾脏和骨髓活检。血清蛋白电泳和免疫固定没有显着差异，但是骨髓涂片的结果与15％浆细胞增多的骨髓瘤的结果一致。然而,肾活检显示弥漫性新月体肾小球肾炎，无免疫复合物或κ/λ链沉积。
方法：最后，患者被诊断为ANCA相关性肾小球肾炎和多发性骨髓瘤双血清阳性.鉴于患者的表现状态，我们开始了低剂量类固醇脉冲治疗，其次是保守的管理。
结果：虽然肺部病变有所改善，肾功能没有恢复以前的状态，促使血液透析开始肾脏替代治疗。自最初诊断以来，抗GBM和抗MPO抗体的水平有所下降。
结论：该病例阐明了ANCA相关性肾小球肾炎和血液系统恶性肿瘤之间复杂的相互作用，并强调了考虑到其多方面的临床表现，需要采用细致入微的治疗策略。
方法：一名79岁的亚裔男子表现为持续性腿部水肿和肾功能不全。他的肾功能迅速下降，血清学检测结果显示抗MPO抗体(54.7IU/mL)和抗GBM抗体(>200IU/mL)的滴度较高.此外,临床特征显示单克隆丙种球蛋白病合并贫血和高球蛋白血症的可能性。我们进行了肾脏和骨髓活检。血清蛋白电泳和免疫固定没有显着差异，但是骨髓涂片的结果与15％浆细胞增多的骨髓瘤的结果一致。然而,肾活检显示弥漫性新月体肾小球肾炎，无免疫复合物或κ/λ链沉积。
方法：最后，患者被诊断为ANCA相关性肾小球肾炎和多发性骨髓瘤双血清阳性.鉴于患者的表现状态，我们开始了低剂量类固醇脉冲治疗，其次是保守的管理。
结果：虽然肺部病变有所改善，肾功能没有恢复以前的状态，促使血液透析开始肾脏替代治疗。自最初诊断以来，抗GBM和抗MPO抗体的水平有所下降。
结论：该病例阐明了ANCA相关性肾小球肾炎和血液系统恶性肿瘤之间复杂的相互作用，并强调了考虑到其多方面的临床表现，需要采用细致入微的治疗策略。
