IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study
Abstract Background 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 th...
Main Authors: | , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
BMC
2020-09-01
|
Series: | BMC Nephrology |
Subjects: | |
Online Access: | http://link.springer.com/article/10.1186/s12882-020-02048-5 |
id |
doaj-a57c112f6c2844159168e3475bd503f2 |
---|---|
record_format |
Article |
spelling |
doaj-a57c112f6c2844159168e3475bd503f22020-11-25T02:49:29ZengBMCBMC Nephrology1471-23692020-09-012111710.1186/s12882-020-02048-5IgM nephropathy complicated by cerebral venous sinus thrombosis: a case studyElizabeth Downie0Jason Diep1Nagendraprasad Sungala2Jeffrey Wong3Department of Renal Medicine, Liverpool HospitalDepartment of Renal Medicine, Liverpool HospitalDepartment of Haematology, Liverpool HospitalDepartment of Renal Medicine, Liverpool HospitalAbstract Background 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. Case presentation 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. Conclusions 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.http://link.springer.com/article/10.1186/s12882-020-02048-5IgM nephropathyCerebral venous sinus thrombosisThrombosisNephrotic syndrome |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Elizabeth Downie Jason Diep Nagendraprasad Sungala Jeffrey Wong |
spellingShingle |
Elizabeth Downie Jason Diep Nagendraprasad Sungala Jeffrey Wong IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study BMC Nephrology IgM nephropathy Cerebral venous sinus thrombosis Thrombosis Nephrotic syndrome |
author_facet |
Elizabeth Downie Jason Diep Nagendraprasad Sungala Jeffrey Wong |
author_sort |
Elizabeth Downie |
title |
IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_short |
IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_full |
IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_fullStr |
IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_full_unstemmed |
IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study |
title_sort |
igm nephropathy complicated by cerebral venous sinus thrombosis: a case study |
publisher |
BMC |
series |
BMC Nephrology |
issn |
1471-2369 |
publishDate |
2020-09-01 |
description |
Abstract Background 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. Case presentation 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. Conclusions 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. |
topic |
IgM nephropathy Cerebral venous sinus thrombosis Thrombosis Nephrotic syndrome |
url |
http://link.springer.com/article/10.1186/s12882-020-02048-5 |
work_keys_str_mv |
AT elizabethdownie igmnephropathycomplicatedbycerebralvenoussinusthrombosisacasestudy AT jasondiep igmnephropathycomplicatedbycerebralvenoussinusthrombosisacasestudy AT nagendraprasadsungala igmnephropathycomplicatedbycerebralvenoussinusthrombosisacasestudy AT jeffreywong igmnephropathycomplicatedbycerebralvenoussinusthrombosisacasestudy |
_version_ |
1724743116376768512 |