Ul Abideen Zain, Sajjad Zoya, Haroon Khan Asna, Mamoon Nadira, Bilal Muhammad, Mujtaba Quadri Khaja Hameeduddin
Nephrology and Renal Transplant, Shifa International Hospital, Islamabad, Pakistan.
Internal Medicine, Shifa International Hospital, Islamabad, Pakistan.
Cureus. 2016 Dec 13;8(12):e929. doi: 10.7759/cureus.929.
Immunoglobulin M nephropathy (IgMN) is characterized by the deposition of immunoglobulin M in a dominant distribution in the renal glomeruli. Primary immunoglobulin M nephropathy is diagnosed after consistent light microscopy (LM), immunofluorescence (IF), electron microscopy (EM) results, and exclusion of known systemic disorders causing immunoglobulin M deposition in the glomeruli. The secondary disease has been reported with a few conditions though it has never been reported with any primary disease of the liver. We report the case of an adolescent male patient who presented with nausea, vomiting, diarrhea, and worsening anasarca. He was found to have nephrotic-range proteinuria that did not respond to conventional corticosteroid treatment. He was subjected to a renal biopsy which revealed a diagnosis of immunoglobulin M nephropathy. His liver function tests were deranged and an ultrasound scan of the abdomen revealed a coarse irregular liver. Workup revealed elevated urine copper excretion and a low ceruloplasmin level. He was diagnosed as a case of Wilson's disease and started on penicillamine and pyridoxine. He was also started on intravenous cyclophosphamide for the corticosteroid-resistant nephrotic syndrome to which he responded remarkably well. His edema settled, proteinuria resolved, and liver functions normalized. Currently, he is in remission and enjoying good health. To the best of our knowledge, we report the first known association between IgM nephropathy and Wilson's disease. It is presently not clear if causation can necessarily be established. This may be the result of defective IgM clearance by the liver or an altered metabolism of the antibody or immune complexes, as with hepatic-associated immunoglobulin M (IgM) nephropathy. Further studies are needed to elucidate the exact mechanism of this disease.
免疫球蛋白M肾病（IgMN）的特征是免疫球蛋白M在肾小球中呈优势分布沉积。原发性免疫球蛋白M肾病是在光镜（LM）、免疫荧光（IF）、电子显微镜（EM）结果一致且排除已知导致肾小球免疫球蛋白M沉积的全身性疾病后确诊的。继发性疾病虽有少数情况被报道，但从未有过与任何原发性肝脏疾病相关的报道。我们报告一例青少年男性患者，他出现恶心、呕吐、腹泻及全身水肿加重。发现他有肾病范围蛋白尿，对传统皮质类固醇治疗无反应。他接受了肾活检，结果诊断为免疫球蛋白M肾病。他的肝功能检查异常，腹部超声扫描显示肝脏粗糙不规则。进一步检查发现尿铜排泄增加和血浆铜蓝蛋白水平降低。他被诊断为威尔逊病，开始使用青霉胺和吡哆醇治疗。他还开始接受静脉注射环磷酰胺治疗对皮质类固醇耐药的肾病综合征，对此他反应良好。他的水肿消退，蛋白尿消失，肝功能恢复正常。目前，他处于缓解期，身体健康。据我们所知，我们报告了首例已知的免疫球蛋白M肾病与威尔逊病之间的关联。目前尚不清楚是否必然能确定因果关系。这可能是肝脏对免疫球蛋白M清除缺陷或抗体或免疫复合物代谢改变的结果，就像与肝脏相关的免疫球蛋白M（IgM）肾病一样。需要进一步研究以阐明这种疾病的确切机制。
