Department of Renal Medicine, Liverpool Hospital, Sydney, NSW, Australia.
Department of Haematology, Liverpool Hospital, Sydney, NSW, Australia.
BMC Nephrol. 2020 Sep 7;21(1):390. doi: 10.1186/s12882-020-02048-5.
IgM nephropathy is a rare disease with variable clinical presentations and is an unusual cause of nephrotic syndrome. Histopathological findings typically include mesangial hypercellularity with IgM and complement deposition, though the spectrum may range from normal glomeruli through to focal and segmental glomerulosclerosis. Thromboembolism is a well recognised complication of nephrotic syndrome, but cerebral venous sinus thrombosis is rarely described.
This is the case of a 23-year-old male presenting with the nephrotic syndrome, whose initial renal biopsy was consistent with minimal change disease. Complete remission was achieved with prednisone, however multiple relapses and steroid dependence prompted re-biopsy, the results of which were more consistent with IgM nephropathy. His last relapse was complicated by cerebral venous sinus thrombosis. He then received rituximab and a weaning course of prednisone to again enter remission.
This case highlights the need to consider IgM nephropathy in the differential diagnosis of nephrotic syndrome. Additionally, it emphasises the risk of thrombosis in patients with severe nephrosis.
IgM 肾病是一种罕见的疾病，临床表现多样，是肾病综合征的不常见病因。组织病理学表现通常包括系膜细胞增生伴 IgM 和补体沉积，尽管其范围可从正常肾小球到局灶节段性肾小球硬化不等。血栓栓塞是肾病综合征的一种公认并发症，但脑静脉窦血栓形成很少见。
这例 23 岁男性患者表现为肾病综合征，其最初的肾活检符合微小病变性疾病。泼尼松治疗后完全缓解，但多次复发和激素依赖促使进行再次肾活检，结果更符合 IgM 肾病。他的最后一次复发并发脑静脉窦血栓形成。随后他接受了利妥昔单抗和逐渐减停泼尼松的治疗，再次进入缓解期。
该病例强调了在肾病综合征的鉴别诊断中需要考虑 IgM 肾病，同时强调了严重肾病患者发生血栓的风险。
