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ORPHA:329903

Immunoglobulin-mediated membranoproliferative glomerulonephritis

high confidenceSubtype of disorder

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

Query health: suspect — Only one of 2 strategies returned hits (phrase).

Publications

327

77.8th percentile

Trials

1

Interventional, condition-specific

Researchers

1,369

Distinct authors in sample

Gene link

DGKE

Strong

Readiness

3/6

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.”

3/6 stages with a signal

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

  1. Gene identifiedPresent

    Strong — DGKE

  2. LiteraturePresent

    327 matched papers (190 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  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)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

327

327 papers have ever been published on this condition (under this name). For scale, breast cancer has over 700,000. Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

327 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

190 in the last 10 years · high confidence · 77.8th percentile (publications denominator)

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 27 July 2026

1 interventional trial — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 76.8th percentile).

high confidence · 76.8th 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

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

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"

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" OR "DGKE"

Recall-expansion terms: DGKE

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: suspect — strategies attempted: phrase, recall-expansion; 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

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