IgM nephropathy – Successful treatment with rituximab

Ahmed, Faheemuddin Azher; El‐Meanawy, Ashraf

doi:10.4103/1319-2442.252917

Cited by 4 publications

(5 citation statements)

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“…Third, with the limited study period and enrolled subject number, it was not possible to analyze response to each treatment in patients in detail. The effectiveness of a specific therapeutic agent, such as rituximab for IgMN, has not been fully investigated yet, although several studies using rituximab as a therapeutic agent for IgMN have been previously reported [ 30 , 31 ]. Since rituximab was used in only one case throughout this cohort, its effect on renal outcome could not be evaluated.…”

Section: Discussionmentioning

confidence: 99%

Renal Outcome of IgM Nephropathy: A Comparative Prospective Cohort Study

Chae

Yoon

Chang

et al. 2021

JCM

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Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis characterized by diffuse deposits of IgM in the glomerular mesangium. However, its renal prognosis remains unknown. We compared renal outcomes of IgMN patients with those of patients with minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or mesangial proliferative glomerulonephritis (MsPGN) from a prospective observational cohort, with 1791 patients undergoing native kidney biopsy in eight hospitals affiliated with The Catholic University of Korea between December 2014 and October 2020. IgMN had more mesangial proliferation and matrix expansion than MsPGN and more tubular atrophy and interstitial fibrosis than MCD. IgMN patients had decreased eGFR than MCD patients in the earlier follow-up. However, there was no significant difference in urine protein or eGFR among all patients at the last follow-up. When IgMN was divided into three subtypes, patients with FSGS-like IgMN tended to have lower eGFR than those with MCD-like or MsPGN-like IgMN but higher proteinuria than MsPGN-like IgMN without showing a significant difference. The presence of hypertension at the time of kidney biopsy predicted ≥20% decline of eGFR over two years in IgMN patients. Our data indicate that IgMN would have a clinical course and renal prognosis similar to MCD, FSGS, and MsPGN

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Section: Discussionmentioning

confidence: 99%

Renal Outcome of IgM Nephropathy: A Comparative Prospective Cohort Study

Chae

Yoon

Chang

et al. 2021

JCM

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“…Our patient was also a carrier of several aHUS common risk variants (CFH promoter SNP, H3 haplotype, and C3 SNP). The CFB mutation and aHUS risk variants identified in our patient jointly represent an increased risk of the dysregulation of the alternative complement pathway causing aHUS [ 21 ]. Therefore, based on the clinical picture, histopathological changes, and the described genetic variants, the patient was diagnosed with aHUS.…”

Section: Discussionmentioning

confidence: 99%

“…To date, one of the most effective treatment regimens for aHUS is plasmatherapy consisting of plasma exchange and/or plasma infusion [ 21 , 35 ]. Our patient received plasma exchange as an empirical treatment for an acute rejection after the first transplant, and this treatment stabilized the graft function.…”

Section: Discussionmentioning

confidence: 99%

Novel Complement Factor B Gene Mutation Identified in a Kidney Transplant Recipient with a Shiga Toxin-Triggered Episode of Thrombotic Microangiopathy

et al. 2022

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Patient: Female, 32-year-old Final Diagnosis: Atypical hemolytic uremic syndrome Symptoms: Kidney injury • loss of the graft • microangiopathic hemolytic anemia • thrombocytopenia Medication: — Clinical Procedure: Dialysis • plasma exchange • plasmapharesis Specialty: Nephrology Objective: Rare disease Background: Atypical hemolytic uremic syndrome (aHUS) is an ultra-rare disorder characterized by over-activation and dys-regulation of the alternative complement pathway. The clinical presentation of the disease comprises thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury. In most cases, aHUS is caused by genetic mutations in components of the alternative complement pathway. The risk of graft loss in patients after kidney transplantation with aHUS is dependent on the type of genetic mutation. Case Report: We present a case of a 32-year-old patient after a second kidney transplantation with atypical hemolytic uremic syndrome, whose clinical manifestation was triggered by the Shiga toxin-producing E. coli infection. Genetic testing revealed a new mutation (p.I342T) in the gene encoding complement factor B (CFB). Since 2 causative variants for aHUS have been described in the same exon of CFB gene, it might be supposed that the p.I342T variant has similar deleterious effects on CFB function. Despite the implemented treatment, graft function deteriorated and the patient had to return to a hemodialysis program and is currently on a waiting list for a third kidney transplant. The presence of the gene variant with increased susceptibility for aHUS and its recurrence after kidney transplantation makes the patient a good candidate for therapy with complement factor C5 inhibitors during and after the planned kidney transplantation. Conclusions: Our case confirms the importance of genetic testing in patients with any sign of thrombotic microangiopathy. The finding of Shiga toxin-induced HUS with typical clinical course should not limit our vigilance of complement-mediated HUS with high risk of renal failure.

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“…Previously second line treatment was with oral cyclophosphamide [5]. More recently, there have been several case studies showing successful treatment with rituximab in native [12] and transplant kidneys [13]. As of yet there are no randomised controlled trials focused on rituximab and its dosing as a treatment option.…”

Section: Discussionmentioning

confidence: 99%

“…As of yet there are no randomised controlled trials focused on rituximab and its dosing as a treatment option. The two case studies using rituximab for IgMN used 375 mg/m 2 4-weekly for 2 doses [13] or 1 g 4-weekly for 2 doses [12]. Betties & Roodnat [13] demonstrated depletion of circulating CD20-positive B cells to less than 0.01 × 10 9 CD20-positive cells/L with just 2 doses of rituximab.…”

Section: Discussionmentioning

confidence: 99%

IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study

et al. 2020

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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.

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IgM nephropathy – Successful treatment with rituximab

Cited by 4 publications

References 0 publications

Renal Outcome of IgM Nephropathy: A Comparative Prospective Cohort Study

Renal Outcome of IgM Nephropathy: A Comparative Prospective Cohort Study

Novel Complement Factor B Gene Mutation Identified in a Kidney Transplant Recipient with a Shiga Toxin-Triggered Episode of Thrombotic Microangiopathy

IgM nephropathy complicated by cerebral venous sinus thrombosis: a case study

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