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.

MO302ACUTE POST-INFECTIOUS GLOMERULONEPHRITIS IN ADULTS: A TUNISIAN SINGLE CENTER EXPERIENCE

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Imen El Meknassi ◽  
Mrabet Sanda ◽  
Guedri Yosra ◽  
Zellema Dorsaf ◽  
Azzabi Awatef ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Kidney Injury ◽  
Acute Glomerulonephritis ◽  
Industrialized Countries ◽  
Single Center Experience ◽  
Nephritic Syndrome ◽  
Stage Renal Disease ◽  
End Stage ◽  
Post Infectious

Abstract Background and Aims Acute post-infectious glomerulonephritis (APIGN) is a reactive immunological disease. Its prevalence in industrialized countries is declining contrasting with developed ones. It is uncommon in adults but the prognosis may be reserved. The aim of our study was to evaluate the epidemiological, clinical and histological features of APIGN as well as its prognosis. Method A retrospective and descriptive study was conducted in our department. Were included all cases of histologically proven APIGN between December 2006 and December 2017. Results We had collected 38 cases. The mean age was 37.7 ± 17.8 years. The sex ratio was 1.92. Twelve (31.6%) patients were diabetic and four of them had already a chronic kidney disease (CKD). APIGN was preceded by an infection in 27 cases with an average interval of 10 ± 5 days. The most common site of infection was the respiratory tract (15 cases). At presentation, 27 patients had nephritic syndrome and 13 had nephrotic-range proteinuria. Hematuria was observed in 97.4%, peripheral edema in 84.2% and hypertension in 73.7% of cases. Most patients (78.9%) had acute kidney injury and 10 (26.3%) patients required dialysis. Renal biopsy had shown benign acute glomerulonephritis in 31 cases and malignant form in 7 cases. An underlying nephropathy was found in 12 cases with mostly a diabetic nephropathy. Corticosteroids were used in 3 cases of benign APIGN and 5 cases of malignant form. During the follow-up, CKD was noted in 14(36.8%) patients including 7(18.4%) patients who progressed to end-stage renal disease. Poor prognostic factors were diabetes, the presence of an underlying nephropathy in the biopsy, acute kidney injury and the need for dialysis. Conclusion The APIGN is uncommon in adults, yet its prognosis may be reserved with progression to CKD.

scholarly journals De novo X-linked Alport syndrome in a 3-year-old girl

2019 ◽  
Vol 12 (7) ◽  
pp. e230183
Author(s):  
Hiroshi Komatsu ◽  
Takeshi Goda ◽  
Kandai Nozu
Keyword(s):  
Alport Syndrome ◽  
De Novo ◽  
Adult Life ◽  
Electron Microscopic ◽  
The Family ◽  
Early Adult Life ◽  
Stage Renal Disease ◽  
End Stage ◽  
Conclusive Diagnosis ◽  
Inherited Kidney Disease

Alport syndrome (AS) is an inherited kidney disease that may lead to end-stage renal disease in early adult life. It is a clinically and genetically heterogeneous nephropathy. The possibility of a patient with haematuria or proteinuria being diagnosed as having AS cannot be excluded even if the patient is female or if the family history is unknown. We report a 3-year-old girl with a de novo frameshift mutation, c.3906delA p.(Gly1303Aspfs*17), in the COL4A5 gene. The significance of the electron microscopic study on the glomerular basement membrane must be emphasised because it is the first step towards the diagnosis of AS. Genetic analysis provides the only conclusive diagnosis of AS, by determining the mode of inheritance and prognosis.

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.

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