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ORPHA:329903

Immunoglobulin-mediated membranoproliferative glomerulonephritis

low confidenceSubtype of disorder

Also known as: Ig-mediated MPGN · Ig-mediated membranoproliferative glomerulonephritis · Immunoglobulin-mediated MPGN

Publications

1,652

Trials

1

Interventional, condition-specific

Researchers

1,369

Distinct authors in sample

Gene link

DGKE

Strong

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A primary form of membranoproliferative glomerulonephritis (MPGN) characterized by deposition in the renal glomeruli of immunoglobulin with complement fractions, especially C3. Clinical presentation may range from nephrotic syndrome and acute kidney injury to asymptomatic proteinuria and hematuria.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (8)

NPHS7 · immune complex mediated membranoproliferative glomerulonephritis · immunoglobulin-mediated MPGN · immunoglobulin-mediated membranoproliferative glomerulonephritis · membranoproliferative glomerulonephritis type I · mesangiocapillary glomerulonephritis type 1 · nephrotic syndrome, type 7 · nephrotic syndrome, type 7, with membranoproliferative glomerulonephritis

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 identifiedPresent

    Strong — DGKE

  2. LiteraturePresent

    1,652 matched papers (1,082 in last 10 years) Source

  3. Phenotype characterisedPresent

    11 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 designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name 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?

Yes — we know a specific gene responsible (DGKE).

GenCC classification: Strong.

Phenotypes (Monarch / HPO)

11

Associated phenotypes · MONDO:0014005

  • Stage 5 chronic kidney disease
  • Nephrotic syndrome
  • Membranoproliferative glomerulonephritis
  • Thrombocytopenia
  • Hemolytic-uremic syndrome

Showing 5 of 11 — 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)

No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.

Open Targets candidates

2

Drugs / clinical candidates · MONDO_0014005

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

1,652

1,652 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.

1,652 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).

1,082 in the last 10 years · low confidence

Phrase hits: 327 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,369

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
    Caravaca-Fontán F7 papers · 2026

    Research Institute "Hospital 12 de Octubre," Madrid, Spain.

    Papers in Europe PMC
  2. 02
    Fakhouri F6 papers · 2026

    Service of Nephrology and Hypertension, Centre Hospitalier Universitaire Vaudois, UNIL, Lausanne, Switzerland.

    Papers in Europe PMC
  3. 03
    Rudnicki M6 papers · 2025

    Department of Internal Medicine IV-Nephrology and Hypertension, Medical University Innsbruck, Innsbruck, Austria.

    Papers in Europe PMC
  4. 04
    Bomback AS5 papers · 2025

    Division of Nephrology, Department of Medicine, Columbia University Irving Medical Center, New York, New York, USA.

    Papers in Europe PMC
  5. 05
    Csuka D5 papers · 2025

    Research Laboratory, 3rd Department of Internal Medicine, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary.

    Papers in Europe PMC
  6. 06
    Miglinas M5 papers · 2026

    Nephrology Center, Santaros Klinikos, Medical Faculty, Vilnius University, Vilnius, Lithuania.

    Papers in Europe PMC
  7. 07
    Prohászka Z5 papers · 2025

    Research Laboratory, 3rd Department of Internal Medicine, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary.

    Papers in Europe PMC
  8. 08
    Remuzzi G5 papers · 2024

    Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Bergamo, Italy; L. Sacco Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy. Electronic address: giuseppe.remuzzi@marionegri.it.

    Papers in Europe PMC
  9. 09
    Sethi S5 papers · 2026

    Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA. sethi.sanjeev@mayo.edu

    Papers in Europe PMC
  10. 10
    Szilágyi Á5 papers · 2025

    Research Laboratory, 3rd Department of Internal Medicine, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary.

    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 · last trial check 28 July 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).

low 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 0 · after dedupe 0 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 0 · dropped 0 · fetched 2026-07-30

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).

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 Immunoglobulin-mediated 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.

("Immunoglobulin-mediated membranoproliferative glomerulonephritis" OR "Ig-mediated MPGN" OR "Ig-mediated membranoproliferative glomerulonephritis" OR "Immunoglobulin-mediated MPGN" OR "NPHS7" OR "immune complex mediated membranoproliferative glomerulonephritis" OR "membranoproliferative glomerulonephritis type I" OR "mesangiocapillary glomerulonephritis type 1" OR "nephrotic syndrome, type 7" OR "nephrotic syndrome, type 7, with membranoproliferative glomerulonephritis") OR ("DGKE" OR "DGKE syndrome" OR "DGKE-related")

Run this search on Europe PMC

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

"Immunoglobulin-mediated membranoproliferative glomerulonephritis" OR "Ig-mediated MPGN" OR "Ig-mediated membranoproliferative glomerulonephritis" OR "Immunoglobulin-mediated MPGN" OR "NPHS7" OR "immune complex mediated membranoproliferative glomerulonephritis" OR "membranoproliferative glomerulonephritis type I" OR "mesangiocapillary glomerulonephritis type 1" OR "nephrotic syndrome, type 7" OR "nephrotic syndrome, type 7, with membranoproliferative glomerulonephritis"

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

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • No synonyms dropped by stoplist
  • No label/synonym collisions with other diseases in this corpus
  • Publication count (1652) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T13:56:41.178Z