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Two Cases of Proliferative Glomerulonephritis With Monoclonal IgG Deposits Treated With Renin Angiotensin Inhibition Alone With Long-term Follow-up.

ABSTRACT:

SUBMITTER: Rosenstock JL

PROVIDER: S-EPMC8343764 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature

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Two Cases of Proliferative Glomerulonephritis With Monoclonal IgG Deposits Treated With Renin Angiotensin Inhibition Alone With Long-term Follow-up.

Rosenstock Jordan L JL Vynnyk Marianna M DeVita Maria V MV D'Agati Vivette D VD

Kidney international reports 20210517 8

PMID: 34386672

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Overexpression of Plasmalemmal Vesicle-Associated Protein-1 Reflects Glomerular Endothelial Injury in Cases of Proliferative Glomerulonephritis with Monoclonal IgG Deposits.

Project description:IntroductionProliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) occasionally presents refractory nephrotic syndrome resulting in poor renal prognosis, but its etiology is not fully elucidated. Given that glomerular endothelial cell (GEC) stress or damage may lead to podocytopathy and subsequent proteinuria, as in thrombotic microangiopathy (TMA), diabetic kidney disease, and focal segmental glomerulosclerosis, we investigated the evidence of glomerular endothelial injury by evaluating the expression of plasmalemmal vesicle-associated protein-1 (PV-1), a component of caveolae in the cases of PGNMID.MethodsWe measured the immunofluorescent PV-1 intensities of 23 PGNMID cases and compared with those of primary membranoproliferative glomerulonephritis (MPGN) (n = 5) and IgA nephropathy (IgAN) (n = 54) cases. PV-1 localization was evaluated with Caveolin-1, and CD31 staining, and the ultrastructural analysis was performed using a low-vacuum scanning electron microscope (LVSEM). To check the association of podocyte injury, we also conducted 8-oxoguanine and Wilms tumor 1 (WT1) double stain. We then evaluated PV-1 expression in other glomerulitis and glomerulopathy such as lupus nephritis and minimal change disease.ResultsThe intensity of glomerular PV-1 expression in PGNMID is significantly higher than that in the other glomerular diseases, although the intensity is not associated with clinical outcomes such as urinary protein levels or renal prognosis. Immunostaining and LVSEM analysis revealed that glomerular PV-1 expression is localized in GECs in PGNMID. 8-oxoguanine accumulation was detected in WT1-positive podocytes but not in PV-1-expressing GECs, suggesting GEC-derived podocyte injury in PGNMID.ConclusionPV-1 overexpression reflects glomerular endothelial injury, which could be associated with podocyte oxidative stress in PGNMID cases.

| S-EPMC9831946 | biostudies-literature

An update of proliferative glomerulonephritis with monoclonal immunoglobulin deposits.

Project description:As aging increases, monoclonal gammopathy is becoming more common and monoclonal gammopathy of renal significance (MGRS) is gaining attention due to frequent renal involvement. Within MGRS, proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a special category. The disease was first described in 2004 and the research history on it is relatively short. Compared with other MGRS, the detection rate of circulating clones is lower in patients with PGNMID, which is easy to miss and misdiagnose in clinical work. In this review, the etiology and clinical features of PGNMID are discussed. It is noted that PGNMID is associated not only with MGRS, but also with malignancy, infection and other factors. PGNMID is not a disease exclusive to the elderly-young people can also develop this disease. Due to the low detection rate of circulating clones in most patients, confirmation of the disease needs to be combined with renal pathology, which emphasizes the importance of completing light and heavy chain subtype staining. Treatment options for patients with PGNMID differ by etiology. For MGRS-associated PGNMID, the current treatment is primarily empirical and more research evidence is needed to fill the treatment gap.

| S-EPMC9155251 | biostudies-literature

KM55 Monoclonal Antibody and IgA Variant of Proliferative Glomerulonephritis With Monoclonal Ig Deposits.

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Daratumumab in Patients With Bortezomib-Refractory Proliferative Glomerulonephritis With Monoclonal Immunoglobulin Deposits.

Project description: Not available

| S-EPMC8343787 | biostudies-literature

Efficacy of Immunomodulatory Drugs in Combination With Dexamethasone in Proliferative Glomerulonephritis With Monoclonal Immunoglobulin Deposits

Project description:Introduction Immunomodulatory drugs (IMiDs) plus dexamethasone are effective for plasma cell dyscrasias, but the treatment efficacy of IMiD in proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) has been rarely reported. Methods We retrospectively analyzed the clinicopathologic data of 64 patients with PGNMID (steroid, IMiD, and bortezomib and dexamethasone/Rituximab [BD/RTX] groups) from January 1, 2010 to December 31, 2020, at the National Clinical Research Center of Kidney Disease in Nanjing. The prognosis of patients receiving different treatment regimens were compared. Factors potentially affecting renal prognosis and renal response were evaluated. Results Twenty-eight, 26 and 10 PGNMID patients were divided into IMiD group, steroid group and BD/RTX group respectively. The rate of serum M protein detection was significantly lower in the steroid group than in the other 2 groups. Renal remission (P = 0.001 and P = 0.03, respectively) rates and renal complete remission (CR) (P = 0.001 and P = 0.01, respectively) rates were significantly higher in the IMiD and BD/RTX groups than in the steroid group at the last follow-up. Multivariate logistic analysis identified that hypertension and high serum creatinine (SCr) levels (>1.24 mg/dl) decreased renal remission, whereas low C3 levels, IMiD and BD/RTX treatments were positively associated with renal remission. Multivariate Cox analysis identified IgG3 in renal tissue and high SCr levels as poor renal prognostic indicators. Severe adverse events were more common in the IMiD and BD/RTX groups than in the steroid group (P = 0.072 and P = 0.035, respectively). Conclusion Our results suggest that IMiDs plus dexamethasone is effective for achieving renal remission in PGNMID patients. Graphical abstract

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Post-infectious Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Deposits Associated with Complement Factor H Mutation.

Project description:A 55-year-old man developed rapidly progressive glomerulonephritis and nephrotic syndrome. A kidney biopsy specimen showed diffuse proliferative and crescentic glomerulonephritis with monoclonal IgG1κ, humps, and nephritis-associated plasmin receptor, indicating infection-associated proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID). Despite dialysis-dependent renal failure, symptomatic therapy resulted in spontaneous recovery of the renal function, mimicking post-infectious glomerulonephritis (PIGN). A heterozygous complement factor H mutation was detected by comprehensive genetic testing of alternative pathway regulatory genes, which might lead to persistent infection-triggered alternative pathway activation and account for severe glomerulonephritis. Post-infectious PGNMID and PIGN might share common clinical presentations and pathogenesis related to the complement pathway.

| S-EPMC5457925 | biostudies-literature

Daratumumab for Bortezomib-Refractory Proliferative Glomerulonephritis With Monoclonal Immunoglobulin Deposits in Kidney Allograft: A Case Report.

Project description:Despite treatment with immunosuppressive or clone-targeted chemotherapy, patients with proliferative glomerulonephritis with monoclonal immunoglobulin deposit (PGNMID) frequently progress into end-stage kidney failure, and early recurrence of PGNMID after kidney transplantation is common. The standard management of PGNMID has been unclear, currently based on data from small cohorts, which requires a need for additional therapeutic regimens in this disease. A human IgG monoclonal antibody that targets CD38 (daratumumab) was recently identified as a potential therapeutic option for treating PGNMID. To date, rare data on the application of daratumumab in patients with PGNMID after kidney transplantation have been reported. Herein, we first described a unique patient with recurrent PGNMID in kidney allograft who was treated with daratumumab after not responding to bortezomib-based regimens. Daratumumab was shown to successfully reduce proteinuria with stabilizing kidney function and was well-tolerated in this patient, which supports that daratumumab appears to be a viable option for treatment-resistant PGNMID.

| S-EPMC11786820 | biostudies-literature

Case report: A case of proliferative glomerulonephritis with monoclonal kappa-light chain deposits treated with daratumumab combination therapy.

Project description:IntroductionProliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a chronic glomerular disease caused by monoclonal gammopathy. IgG (mainly IgG3) is the most commonly involved isotype of PGNMID. Here we illustrated a novel medication regimen for the rare variant of PGNMID with deposition of monoclonal immunoglobulin light chain only (PGNMID-LC). Daratumumab has been proved effective in the treatment of plasma cell myeloma while its effect for PGNMID-LC has rarely been reported.MethodsA daratumumab combination therapy (D-VCd regimen, specifically are daratumumab + dexamethasone + bortezomib + cyclophosphamide) was adopted to treat a patient diagnosed with PGNMID-LC.ResultsThe utility of D-VCd regimen showed a favorable effect in this patient. After the fixed course, his clinical symptom, laboratory parameters, neoplastic plasma cells clonity all restored to normal range, and no obvious disease progression was observed throughout the treatment. After a follow up of 14 months, no significant renal or hematological disease progression has been observed.ConclusionThis case underscores the utility of D-VCd regimen in treatment of PGNMID-LC, and it's inferred that daratumumab regimen has clinical effects in the disease primarily through targeting tumor clonity. However, data on the use of daratumumab (either in monotherapy or in combination) in clinical trials of PGNMID-LC is currently so limited that that more experiments are needed to support the inference.

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Atypical Infection-Related Glomerulonephritis With "Masked" IgG-Kappa Crystalline Hump-Like Deposits.

Project description: Not available

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Renin-angiotensin system blockade is renoprotective in immune complex-mediated glomerulonephritis.

Project description:Blockade of the renin-angiotensin system is renoprotective in a variety of chronic nephropathies, but the direct effect of such treatment in active, immune complex-mediated glomerulonephritis is unknown. This study investigated the short- and long-term effects of an angiotensin-converting enzyme inhibitor (enalapril) and an angiotensin II type 1 receptor blocker (losartan) in thymic stromal lymphopoietin transgenic (TSLPtg) mice, which develop mixed cryoglobulinemia and severe cryoglobulinemia-associated membranoproliferative glomerulonephritis. Enalapril and losartan each reduced hypertension, proteinuria, glomerular extracellular matrix deposition, and mesangial cell activation in TSLPtg mice. These renoprotective effects were not observed with hydralazine treatment, despite a similar antihypertensive effect. Treatment with enalapril or losartan also decreased renal plasminogen activator inhibitor-1 in TSLPtg mice, assessed by immunohistochemistry and quantitative real-time reverse transcriptase-PCR. None of the treatments affected immune complex deposition or macrophage infiltration. Overall, enalapril- and losartan-treated TSLPtg mice survived significantly longer than untreated TSLPtg mice. These studies demonstrate that angiotensin blockade may provide renoprotective benefits, independent of its BP-lowering effect, in the treatment of active immune complex-mediated glomerulonephritis.

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