成人表现为新月体性肾小球肾炎和肾病综合征的免疫球蛋白 M 肾病病例报告。
A case report of immunoglobulin M nephropathy manifesting as crescentic glomerulonephritis and nephrotic syndrome in an adult.
机构信息
Department of Internal Medicine, Division of Nephrology, NHIS Ilsan Hospital, Goyang, Gyeonggi-do, 10444, Republic of Korea.
Department of Pathology, NHIS Ilsan Hospital, Goyang, Gyeonggi-do, 10444, Republic of Korea.
出版信息
BMC Nephrol. 2019 Aug 27;20(1):335. doi: 10.1186/s12882-019-1528-2.
BACKGROUND
The nature of immunoglobulin M (IgM) nephropathy has been controversial for a long time, but it is now considered an independent disease like immunoglobulin A nephropathy. IgM nephropathy has been known to have various clinical manifestations ranging from asymptomatic hematuria and/or proteinuria to nephrotic syndrome. Recently, one case of IgM nephropathy manifesting as crescentic glomerulonephritis (GN) was reported in a child.
CASE PRESENTATION
We experienced a case of IgM nephropathy that manifested clinically as nephritic and nephrotic syndrome with pathologically confirmed crescentic GN in a 30-year-old woman. We administered a calcineurin inhibitor and corticosteroids to treat the ongoing nephrotic syndrome after remission of crescentic GN. As a result, her proteinuria was significantly reduced and edema improved.
CONCLUSIONS
We described a case of IgM nephropathy in an adult patient who initially developed crescentic GN with nephritic and nephrotic syndrome. This case report could contribute to a deeper understanding of IgM nephropathy.
背景
免疫球蛋白 M（IgM）肾病的性质长期以来一直存在争议，但现在它被认为是一种与免疫球蛋白 A 肾病一样的独立疾病。IgM 肾病的临床表现多种多样，从无症状血尿和/或蛋白尿到肾病综合征不等。最近，有报道称儿童 IgM 肾病表现为新月体性肾小球肾炎（GN）。
病例介绍
我们遇到了一例 IgM 肾病，表现为临床肾病综合征和病理证实的新月体性 GN 的 30 岁女性。我们在新月体性 GN 缓解后使用钙调磷酸酶抑制剂和皮质类固醇治疗持续的肾病综合征。结果，她的蛋白尿明显减少，水肿改善。
结论
我们描述了一例成人 IgM 肾病患者，最初表现为新月体性 GN 伴肾病综合征和肾病综合征。本病例报告有助于加深对 IgM 肾病的认识。
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