scholarly journals Recurrence of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Deposits with a Striated Ultrastructure

Nephron ◽  
2020 ◽  
pp. 1-6
Author(s):  
Tomoko Namba-Hamano ◽  
Takayuki Hamano ◽  
Ryoichi Imamura ◽  
Yutaka Yamaguchi ◽  
Masahiro Kyo ◽  
...  
Keyword(s):  
Striated Muscle ◽  
Pulse Therapy ◽  
Early Recurrence ◽  
Proliferative Glomerulonephritis ◽  
Monoclonal Immunoglobulin ◽  
Legionella Pneumonia ◽  
Allograft Biopsy ◽  
Dense Deposits ◽  
Subendothelial Space ◽  
One Year

A 64-year-old man with nephrotic syndrome was admitted to another hospital where his renal biopsy revealed membranoproliferative glomerulonephritis (MPGN) with monoclonal immunoglobulin (Ig) G, subclass 1, κ light chain (IgG1κ) deposition on immunofluorescence (IF). Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) was suspected due to monoclonal IgG1κ deposits and the absence of hematological abnormalities. However, the typical PGNMID phenotype was not observed by electron microscopy. Instead, an organized and striated muscle-like structure was observed in the subendothelial space. Since a 2-year treatment with immunosuppressants did not improve his proteinuria, a second biopsy was performed at our hospital. It showed an MPGN-like phenotype; however, monoclonal Ig deposits on IF were no longer observed. One year after the second biopsy, he developed ESRD. Thus, he underwent living kidney transplantation from his wife. Allograft biopsy was performed as proteinuria was observed 3 months after transplantation, which again showed an MPGN-like phenotype with monoclonal IgG1κ deposits. The observed electron-dense deposits were similar to those in the native biopsies. Accordingly, the patient was diagnosed with recurrent MPGN. Adding methylprednisolone pulse therapy to conventional immunosuppressants did not improve the patient’s renal function or proteinuria. He died of Legionella pneumonia 8 months after transplantation. Considering the patient’s histological findings of MPGN with monoclonal IgG1κ deposits and early recurrence of glomerulonephritis after transplantation, he was diagnosed with PGNMID with novel electron-dense deposits.

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a nephrologist perspective

2019 ◽  
Author(s):  
Frank Bridoux ◽  
Vincent Javaugue ◽  
Samih H Nasr ◽  
Nelson Leung
Keyword(s):  
Cell Clone ◽  
Early Recurrence ◽  
Proliferative Glomerulonephritis ◽  
Monoclonal Immunoglobulin ◽  
Electron Microscopic ◽  
Nephritic Syndrome ◽  
Microscopic Studies ◽  
Immunoglobulin Deposits ◽  
Stage Renal Disease ◽  
End Stage

AbstractProliferative glomerulonephritis (GN) with monoclonal immunoglobulin deposits (PGNMIDs) is a recently described entity among the spectrum of monoclonal gammopathy of renal significance (MGRS). The disease is renal limited and manifests with chronic glomerular disease, altered renal function and albuminuria, sometimes in the nephrotic range. Acute nephritic syndrome is rare. PGNMID occurs mostly in the sixth decade, but it may affect young adults. Histologically, PGNMID is characterized predominantly by membranoproliferative GN and less frequently by diffuse endocapillary GN, mesangioproliferative GN or atypical membranous GN. Immunofluorescence and electron microscopic studies are the cornerstone of diagnosis, showing granular deposits involving glomeruli only, and composed of monotypic immunoglobulin G (IgG), with a single heavy chain subclass (most commonly IgG3) and light chain (LC) restriction (usually κ), admixed with complement deposits. PGNMID variants with monotypic LC-only, IgA or IgM deposits are uncommon. Ultrastructurally, deposits are amorphous with predominant subendothelial and mesangial distribution. PGNMID should be distinguished from type 1 cryoglobulinemic GN and immunotactoid GN, which share some common pathological features. Contrary to other MGRS lesions, the rate of detection of the nephrotoxic monoclonal Ig in the serum or urine, and of an abnormal bone marrow B-cell clone, is only ∼30%. Renal prognosis is poor, with progression to end-stage renal disease in 25% of patients within 30 months and frequent early recurrence on the renal allograft. The pathophysiology of PGNMID is unclear and its treatment remains challenging. However, recent studies indicate that clone-targeted chemotherapy may significantly improve renal outcomes, opening future perspectives for the management of this rare disease.

scholarly journals Nephrotic Syndrome Secondary to Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits of Lambda Light Chain

2014 ◽  
Vol 2014 ◽  
pp. 1-6
Author(s):  
Seongseok Yun ◽  
Beth L. Braunhut ◽  
Courtney N. Walker ◽  
Waheed Bhati ◽  
Amy N. Sussman ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Light Chain ◽  
Hematologic Malignancies ◽  
Proliferative Glomerulonephritis ◽  
Lambda Light Chain ◽  
Monoclonal Immunoglobulin ◽  
Subendothelial Space ◽  
History Of ◽  
Immunoglobulin Deposits ◽  
Refractory Nephrotic Syndrome

We describe a rare case of a 46-year-old woman with history of refractory nephrotic syndrome and hypertension who presented with worsening proteinuria and kidney function. Work-up for both autoimmune and infectious diseases and hematologic malignancies including multiple myeloma were negative. Kidney biopsy demonstrated glomerular sclerotic change with lambda light chain deposits in the subendothelial space, which is consistent with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID). The patient was treated with bortezomib and dexamethasone without clinical improvement and eventually became hemodialysis dependent.

Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits is associated with high rate of early recurrence in the allograft

2018 ◽  
Vol 94 (1) ◽  
pp. 159-169 ◽  
Author(s):  
Samar M. Said ◽  
Fernando G. Cosio ◽  
Anthony M. Valeri ◽  
Nelson Leung ◽  
Sanjeev Sethi ◽  
...  
Keyword(s):  
Immunoglobulin G ◽  
Early Recurrence ◽  
Proliferative Glomerulonephritis ◽  
High Rate ◽  
Monoclonal Immunoglobulin

scholarly journals Efficacy of methylprednisolone therapy in atypical Rolandic epilepsy with heterozygous RELN mutation

2019 ◽  
Author(s):  
Xiaoyue Hu ◽  
Miao Jing ◽  
Ying Hua
Keyword(s):  
Corticosteroid Therapy ◽  
Therapeutic Option ◽  
Gene Mutations ◽  
Pulse Therapy ◽  
High Dose ◽  
Rolandic Epilepsy ◽  
Whole Exome ◽  
Suitable Treatment ◽  
One Year ◽  
Intravenous Methylprednisolone Pulse

Abstract Background: Atypical Rolandic epilepsy, also known as benign childhood epilepsy with centrotemporal spikes (BECTS) variant is defined by the appearance of severe neuropsychological impairments and refractory epilepsy. The etiology of the disease remains unclear, and recent studies indicated that it is related to several gene mutations. The suitable treatment is also need to be further explored. Here, we present the case of a 9-year-old boy with BECTS variant found to have heterozygous RELN mutation, responded well to the corticosteroid therapy. Case presentation: A 9-year-old male patient with atypical benign Rolandic epilepsy was successfully treated with high-dose intravenous methylprednisolone pulse therapy (15 mg/kg daily for 3 consecutive days, and the infusion was repeated three times with a 4-day interval between each course). The treatment improved his electroencephalogram (EEG) and cognitive performance, reduced seizure frequency, and the effect maintained for one year of follow-up. Whole-exome sequencing (WES) revealed a meaningful heterozygous missense mutation in the RELN gene. Conclusion: We conclude that corticosteroid therapy should be considered as a therapeutic option in patients with BECTS variant who are also found to harbour RELN mutations.

scholarly journals Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits in a young woman: A case report

2021 ◽  
Vol 9 (10) ◽  
pp. 2357-2366
Author(s):  
Zi-Gan Xu ◽  
Wei-Long Li ◽  
Xi Wang ◽  
Shu-Yuan Zhang ◽  
Ying-Wei Zhang ◽  
...  
Keyword(s):  
Case Report ◽  
Young Woman ◽  
Immunoglobulin G ◽  
Proliferative Glomerulonephritis ◽  
Monoclonal Immunoglobulin
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