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ORPHA:329918

C3 glomerulopathy

medium confidenceSubtype of disorder

Also known as: Non-Ig-mediated MPGN · Non-Ig-mediated membranoproliferative glomerulonephritis · Non-immunoglobulin-mediated MPGN · Non-immunoglobulin-mediated membranoproliferative glomerulonephritis

Publications

2,033

89.9th percentile

Trials

19

Interventional, condition-specific

Researchers

1,266

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A form of primary membranoproliferative glomerulonephritis characterized by the presence in renal biopsy samples of a glomerulonephritis with sole (or at least ) glomerular immunofluorescence staining for C3. Non-specific alterations or proliferative patterns with C3- glomerular staining are also possible. Based upon electron microscopic findings, C3 glomerulopathy (C3G) may be further classified as C3 glomerulonephritis (C3GN) or Dense deposit disease (DDD).

How rare: 1-9 / 1 000 000 — roughly one to nine people per million. In a city the size of Kolkata, perhaps a few dozen.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (5)

C3G · non-Ig-mediated MPGN · non-Ig-mediated membranoproliferative glomerulonephritis · non-immunoglobulin-mediated MPGN · non-immunoglobulin-mediated 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 identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    2,033 matched papers (1,707 in last 10 years) Source

  3. Phenotype characterisedPresent

    39 HPO annotations (e.g. Decreased circulating complement factor H concentration; Depletion of components of the alternative complement pathway; Glomerular subendothelial electron-dense deposits) Source

  4. Animal modelNot found

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

  5. Orphan designationPartial

    6 EMA designations (none yet with FDA orphan-indication approval) — e.g. Complement factor H Source

  6. Interventional trialPresent

    19 matched on ClinicalTrials.gov (7 recruiting in sample)

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)

39

Associated phenotypes · MONDO:0018013

  • Decreased circulating complement factor H concentration
  • Depletion of components of the alternative complement pathway
  • Glomerular subendothelial electron-dense deposits
  • Chronic kidney disease
  • Thickened glomerular basement membrane

Showing 5 of 39 — 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)

6

Designations · no FDA orphan-indication approval yet

  • EMA Complement factor HTreatment of C3 glomerulopathy · 28/06/2024 · PositiveEMA designation
  • EMA Poly(oxy-1,2-ethanediyl), alpha-hydro-omega-hydroxy-,15,15'-diester with N-acetyl-L-isoleucyl-L-cysteinyl-L-valyl-1-methyl-L-tryptophyl-L-glutaminyl-L-alpha-aspartyl-L-tryptophylglycyl-L-alanyl-L-histidyl-L-arginyl-L-cysteinyl-L-threonyl-2-[2-(2-aminoethoxy)ethoxy]acetyl-N6-carboxy-L-lysinamide cyclic (2.fwdarw.12)-(disulfide); where two identical synthetic peptide domains are covalently linked at the ends of the polyethylene glycol chain (pegcetacoplan)Treatment of C3 glomerulopathy · 21/08/2019 · PositiveEMA designation
  • EMA avacopanTreatment of C3 glomerulopathy · 20/06/2017 · PositiveEMA designation
  • EMA S3,S13-cyclo(D-tyrolsyl-L-isoleucyl-L-cysteinyl-L-valyl-1-methyl-L-tryptophyl-L-glutaminyl-L-aspartyl-L-tryptophyl-N-methyl-L-glycyl-L-alanyl-L-histidyl-L-arginyl-L-cysteinyl-N-methyl-L-isoleucinamide)Treatment of C3 glomerulopathy · 17/02/2016 · PositiveEMA designation
  • EMA (4-{(2S,4S)-4-ethoxy-1-[(5-methoxy-7-methyl-1H-indol-4-yl)methyl]piperidin-2-yl}benzoic acid-hydrogen chloride(1/1))Treatment of C3 glomerulopathy · 14/12/2018 · PositiveEMA designation
  • EMA (2S,4R)-1-(2-(3-acetyl-5-(2-methylpyrimidine-5-yl)-1H-indazol-1-yl)acetyl)-N-(6-bromopyridine-2-yl)-4-fluoropyrrolidine-2-carboxamideTreatment of C3 glomerulopathy · 21/03/2018 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

9

Drugs / clinical candidates · MONDO_0018013

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

2,033

2,033 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.

2,033 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).

1,707 in the last 10 years · medium confidence · 89.9th percentile (publications denominator)

Phrase hits: 1,829 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,266

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 F11 papers · 2026

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

    Papers in Europe PMC
  2. 02
    Nester CM10 papers · 2026

    Division of Nephrology, Department of Medicine, University of Iowa, Iowa City, IA.

    Papers in Europe PMC
  3. 03
    Vivarelli M10 papers · 2026

    Laboratory of Nephrology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy. marina.vivarelli@opbg.net.

    Papers in Europe PMC
  4. 04
    Remuzzi G9 papers · 2026

    Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, Bergamo, Italy.

    Papers in Europe PMC
  5. 05
    Bomback AS8 papers · 2026

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

    Papers in Europe PMC
  6. 06
    Fakhouri F7 papers · 2026

    Service of Nephrology and Hypertension, Lausanne University Hospital and University of Lausanne, Switzerland.

    Papers in Europe PMC
  7. 07
    Pickering MC7 papers · 2026

    Department of Immunology and Inflammation, Imperial College London, London, United Kingdom.

    Papers in Europe PMC
  8. 08
    Chauvet S6 papers · 2026

    Inflammation, Complement and Cancer Team, Centre de Recherche des Cordeliers, Sorbonne Université, Inserm, Université Paris Cité, Paris, France.

    Papers in Europe PMC
  9. 09
    Meuleman MS6 papers · 2026

    Inflammation, Complement and Cancer Team, Centre de Recherche des Cordeliers, Sorbonne Université, Inserm, Université Paris Cité, Paris, France.

    Papers in Europe PMC
  10. 10
    Noris M6 papers · 2026

    Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Centro Anna Maria Astori, Science and Technology Park Kilometro Rosso, Bergamo, Italy.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

19

interventional trials for this specific condition

19 interventional trials matched this specific condition name; 7 currently recruiting in our sample.

Data as of 11 September 2026

19 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 94.6th percentile).

medium confidence · 94.6th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

6 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

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 10 · after dedupe 10 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 10 · 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).

Uncertain / not reviewed (10)

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 C3 glomerulopathy — 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.

("C3 glomerulopathy" OR "Non-Ig-mediated MPGN" OR "Non-Ig-mediated membranoproliferative glomerulonephritis" OR "Non-immunoglobulin-mediated MPGN" OR "Non-immunoglobulin-mediated membranoproliferative glomerulonephritis") OR ("C3 syndrome" OR "C3-related")

Run this search on Europe PMC

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

"C3 glomerulopathy" OR "Non-Ig-mediated MPGN" OR "Non-Ig-mediated membranoproliferative glomerulonephritis" OR "Non-immunoglobulin-mediated MPGN" OR "Non-immunoglobulin-mediated 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: 19 interventional · 6 observational · 2 expanded access. Only interventional studies enter the trial headline.

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: C3G

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 2026-07-27T13:57:04.785Z