IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study
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CASE REPORT
Open Access
IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study Elizabeth Downie1* , Jason Diep1, Nagendraprasad Sungala2 and Jeffrey Wong1
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. Keywords: IgM nephropathy, Cerebral venous sinus thrombosis, Thrombosis, Nephrotic syndrome
Learning points Always consider thromboses as a cause of unusual
symptoms when patients have severe nephrosis Have a low threshold for repeating a renal biopsy if
the clinical picture is not consistent with the initial histological diagnosis IgM nephropathy is a rare condition with variable presentation, ranging from microscopic haematuria through to the nephrotic syndrome
Background Immunoglobulin M nephropathy (IgMN) is a rare disease with variable presentation, from microscopic haematuria through to the nephrotic syndrome (NS) [1]. A recent large case series showed IgMN to have a * Correspondence: [email protected] 1 Department of Renal Medicine, Liverpool Hospital, Sydney, NSW, Australia Full list of author information is available at the end of the article
prevalence of 1.8% of all native kidney biopsies [2]. The typical findings of IgMN on light microscopy (LM) include mesangial hypercellularity which may be mild, with immunofluorescence (IF) showing IgM as the sole or dominant immunoglobulin in the mesangium of the glomeruli in a diffuse and global pattern [3]. Associated with the immunoglobulin M (IgM) deposition is complement deposition, specifically complement component 3 (C3) [4]. However, the histological findings can vary from no glomerular abnormalities through to mesangial hyperplasia associated with segmental and global sclerosis [5]. This variability has led to ongoing conjecture about how IgMN fits into the spectrum of glomerulonephritis (GN) [1]. This case demonstrates the importance of re-biopsy and considering IgMN in the
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