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primary membranoproliferative glomerulonephritis (MPGN) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

primary membranoproliferative glomerulonephritis

MONDO:0018904

Also known as:MPGNMesangiocapillary glomerulonephritismembranoproliferative glomerulonephritis

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Overview

A rare glomerular disease characterized by a pattern of glomerular injury on kidney biopsy with characteristic light microscopic changes: mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (GBM). On the basis of immunofluorescence (IF) the disorder is divided into C3 glomerulopathy (C3G) or immunoglobulin-mediated membranoproliferative glomerulonephritis. Through electron microscopy C3G is further divided into Dense deposit disease, with highly electrondense deposits in the glomerular basement membrane, and C3 glomerulonephritis, with mesangial, intramembranous, subendothelial and subepithelial deposits. Secondary causes (autoimmune, infectious, malignancies) are excluded.

TreatmentsNo approved treatments · 1 in pipeline

Clinical Trials7 registered · 4 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include very common findings: Membranoproliferative glomerulonephritis; and common findings: Reduced kidney function (renal insufficiency), Protein in the urine (proteinuria), Nephrotic syndrome, and Hypertension and others. 16 total HPO annotations.

Organ System	Phenotype Count	Example Features
Kidneys and urinary system	9	Membranoproliferative glomerulonephritis, Reduced kidney function (renal insufficiency), Protein in the urine (proteinuria)

Diagnosis

Biomarker and diagnostic research for primary membranoproliferative glomerulonephritis has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for primary membranoproliferative glomerulonephritis. An additional 1 compound holds orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for primary membranoproliferative glomerulonephritis, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for primary membranoproliferative glomerulonephritis. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Prognosis

Phenotype severity distribution: 1 very common feature, 9 common features.

Estimated prevalence: 1-5 in 10,000 (Uncommon).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

7 clinical trials registered, 4 recruiting. Interventions under study include drug therapy, other interventions, and procedural interventions. Pipeline includes 2 PHASE3, 2 PHASE2, 2 NA. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT05809531](https://clinicaltrials.gov/study/NCT05809531)

Data assembled from 6 of 12 sources · Last updated Sep 17, 2026, 8:25 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:54370

GARD

Genetic and Rare Diseases Info Center

GARD:11982

Questions for Your Care Team

→Are any of the 7 active clinical trials a good fit for my situation?
→What is the current development status of recombinant human minibody against complement component C5, and when might it become available?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Patient case studies	61	46%
Research summaries	29	22%
Disease patterns and progression	25	19%
Laboratory research	6	5%
Other research	4	3%
Clinical study results	4	3%
Testing and diagnosis research	3	2%
New treatment approaches	1	1%

Kisho

primary membranoproliferative glomerulonephritis

Overview

Clinical Presentation

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Clinical Trials

Recent Publications

Latest News & Developments

Immune-complex-mediated membranoproliferative glomerulonephritis with linear IgG staining resembling atypical anti-glomerular basement membrane disease: a case report.

Initial treatment patterns of primary membranoproliferative glomerulonephritis in Japan (2017-2021): an updated analysis based on nationwide personal clinical records.

primary membranoproliferative glomerulonephritis

Overview

Clinical Presentation

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Clinical Trials

Recent Publications

Latest News & Developments

Immune-complex-mediated membranoproliferative glomerulonephritis with linear IgG staining resembling atypical anti-glomerular basement membrane disease: a case report.

Initial treatment patterns of primary membranoproliferative glomerulonephritis in Japan (2017-2021): an updated analysis based on nationwide personal clinical records.