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ORPHA:54370

Primary membranoproliferative glomerulonephritis

medium confidenceDisorder

Also known as: Mesangiocapillary glomerulonephritis · Primary MPGN

Publications

792

71.4th percentile

Trials

1

Interventional, condition-specific

Researchers

1,234

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

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.

How rare: 1-5 / 10 000 — about one to five people per ten thousand (still uncommon, but less ultra-rare).

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (1)

MPGN

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

4/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    792 matched papers (242 in last 10 years) Source

  3. Phenotype characterisedPresent

    66 HPO annotations (e.g. Stage 5 chronic kidney disease; Nephrotic syndrome; Membranoproliferative glomerulonephritis) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPartial

    1 EMA designation (none yet with FDA orphan-indication approval) — e.g. recombinant human minibody against complement component C5 Source

  6. Interventional trialPresent

    1 matched on ClinicalTrials.gov

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

66

Associated phenotypes · MONDO:0018904

  • Stage 5 chronic kidney disease
  • Nephrotic syndrome
  • Membranoproliferative glomerulonephritis
  • Decreased circulating complement factor H concentration
  • Depletion of components of the alternative complement pathway

Showing 5 of 66 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

1

Designation · no FDA orphan-indication approval yet

  • EMA recombinant human minibody against complement component C5Treatment of primary membranoproliferative glomerulonephritis · 27/10/2011 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

3

Drugs / clinical candidates · MONDO_0018904

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

792

792 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

792 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

242 in the last 10 years · medium confidence · 71.4th percentile (publications denominator)

Phrase hits: 792 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,234

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Chen J5 papers · 2024

    Xiamen Amoytop Biotech Co., Ltd., Xiamen, China.

    Papers in Europe PMC
  2. 02
    Isaka Y4 papers · 2026

    Department of Nephrology, Osaka University Graduate School of Medicine, Suita, Osaka, Japan.

    Papers in Europe PMC
  3. 03
    Liu J4 papers · 2026

    Department of Nephrology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.

    Papers in Europe PMC
  4. 04
    Nakagawa N4 papers · 2026

    Division of Cardiology and Nephrology, Department of Internal Medicine, Asahikawa Medical University, Asahikawa, Japan. naka-nao@asahikawa-med.ac.jp.

    Papers in Europe PMC
  5. 05
    Fakhouri F3 papers · 2026

    Lausanne University Hospital, University of Lausanne, Lausanne, Switzerland.

    Papers in Europe PMC
  6. 06
    Jiang SH3 papers · 2018

    Department of Renal Medicine, The Canberra Hospital, PO Box 11, Woden, ACT, 2605, Australia.

    Papers in Europe PMC
  7. 07
    Mubarak M3 papers · 2024

    Department of Pathology, Sindh Institute of Urology and Transplantation, Sindh, Karachi 74200, Pakistan. drmubaraksiut@yahoo.com.

    Papers in Europe PMC
  8. 08
    Narita I3 papers · 2023

    Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

    Papers in Europe PMC
  9. 09
    Shen Q3 papers · 2025

    Department of Nephrology, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.

    Papers in Europe PMC
  10. 10
    Walters GD3 papers · 2018

    Department of Renal Medicine, The Canberra Hospital, PO Box 11, Woden, ACT, 2605, Australia. giles.walters@act.gov.au.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

1

interventional trials for this specific condition

1 interventional trial matched this specific condition name; none in our sample are currently recruiting. 116 trials are registered for glomerulonephritis, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

1 interventional trial — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 80.1th percentile).

medium confidence · 80.1th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

1 interventional trials matched after quoted-phrase search and title/condition post-filter.

No currently recruiting studies in the matched set. Open the same search on ClinicalTrials.gov.

Broader category: glomerulonephritis

116

Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.

Worth raising with a clinician. How we count trials.

Recruiting under the broader category

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 1 · after dedupe 1 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 1 · dropped 0 · fetched 2026-07-29

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (1)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Primary membranoproliferative glomerulonephritis — check Associations / patient organisations on that page.

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Primary membranoproliferative glomerulonephritis" OR "Mesangiocapillary glomerulonephritis" OR "Primary MPGN"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Primary membranoproliferative glomerulonephritis" OR "Mesangiocapillary glomerulonephritis" OR "Primary MPGN"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 1 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"glomerulonephritis"

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: MPGN

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 1 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-27T00:57:06.672Z