ORPHA:329931
C3 glomerulonephritis
Publications
1,797
Trials
10
Interventional, condition-specific
Researchers
1,354
Distinct authors in sample
Gene link
C3, CFB, CFH
Definitive
Readiness
4/6
Stages with a signal
Clinical definition (Orphanet)
A histological subtype of C3 glomerulopathy characterized by C3 deposition in renal tissue in the absence or near-absence of immunoglobulin deposits, in a patient with the classic clinical features of glomerulonephritis and the electron microscopic findings of predominant subendothelial, occasionally subepithelial (so-called ''humps''), and intramembranous deposits, but without the typical electron-dense deposits of dense deposit disease.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0013892
- OMIM:614809
- UMLS:C4055342
- NCIT:C123043
Additional Mondo synonyms (2)
complement-mediated membranoproliferative glomerulonephritis · nephropathy due to CFHR5 deficiency
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.
- Gene identifiedPresent
Definitive — C3, CFB, CFH, CFHR5, CFI
- LiteraturePresent
1,797 matched papers (1,289 in last 10 years) Source
- Phenotype characterisedPresent
9 HPO annotations (e.g. Glomerulonephritis; Stage 5 chronic kidney disease; Mesangial matrix expansion) Source
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- Interventional trialPresent
10 matched on ClinicalTrials.gov (1 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?
Yes — we know a specific gene responsible (C3, CFB, CFH…).
GenCC classification: Definitive.
Phenotypes (Monarch / HPO)
9
Associated phenotypes · MONDO:0013892
- Glomerulonephritis
- Stage 5 chronic kidney disease
- Mesangial matrix expansion
- Thickening of glomerular capillary wall
- Glomerular subendothelial electron-dense deposits
Showing 5 of 9 — 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_0013892
- AVACOPAN·phase 2
- ECULIZUMAB·phase 1
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
Literature
Is anyone studying this?
1,797
1,797 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.
1,797 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).
1,289 in the last 10 years · low confidence
Phrase hits: 1,230 · MeSH hits: 0
Who's working on it?
1,354
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.
- 01Caravaca-Fontán F7 papers · 2026
Department of Nephrology, Research Institute Hospital 12 de Octubre (imas12), Madrid, Spain.
Papers in Europe PMC - 02Praga M6 papers · 2026
Department of Medicine, Complutense University, Madrid, Spain.
Papers in Europe PMC - 03Sethi S6 papers · 2026
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Papers in Europe PMC - 04Fervenza FC4 papers · 2026
Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA.
Papers in Europe PMC - 05Emma F3 papers · 2025
Division of Nephrology, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy.
Papers in Europe PMC - 06Lee H3 papers · 2025
Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
Papers in Europe PMC - 07Nagano C3 papers · 2025
Department of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, 650-0017, Japan.
Papers in Europe PMC - 08Nozu K3 papers · 2025
Department of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuo-Ku, Kobe, 650-0017, Japan.
Papers in Europe PMC - 09Ohashi K3 papers · 2026
Department of Human Pathology, Tokyo Medical and Dental University, Tokyo.
Papers in Europe PMC - 10Sawai T3 papers · 2025
Department of Pediatrics, Shiga University of Medical Science, Otsu, Japan.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
10
interventional trials for this specific condition
10 interventional trials matched this specific condition name; 1 currently recruiting in our sample.
Data as of 11 September 2026 · last trial check 28 July 2026
10 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 92.5th percentile).
low confidence · 92.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
10 interventional trials matched after quoted-phrase search and title/condition post-filter.
- NCT04183101·RECRUITING·Evaluation of a Renin Inhibitor, Aliskiren, Compared to Enalapril, in C3 Glomerulopathy
Not reviewed·Conditions: C3 Glomerulopathy · Membranoproliferative Glomerulonephritis · Complement Abnormality · Dense Deposit Disease·Matched via name phrase
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 3 · after dedupe 3 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 3 · 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 (3)
- isrctn·ISRCTN17045863·Not yet recruiting·The National Unified Renal Translational Research Enterprise for biosampling patients with rare kidney disease
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN85816718·No longer recruiting·A study to evaluate the effectiveness and safety of obinutuzumab in Chinese participants with Class III or IV lupus nephritis
skipped — LLM skipped (--skip-llm)
- isrctn·ISRCTN84672048·No longer recruiting·SM101 In systemic lupus erythematosus patients with or without a history of lupus nephritis
skipped — LLM skipped (--skip-llm)
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 glomerulonephritis — check Associations / patient organisations on that page.
India — NPRD
Last verified 2026-07-26This 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 glomerulonephritis" OR "complement-mediated membranoproliferative glomerulonephritis" OR "nephropathy due to CFHR5 deficiency") OR ("C3 syndrome" OR "C3-related" OR "CFB syndrome" OR "CFB-related" OR "CFH syndrome" OR "CFH-related")ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"C3 glomerulonephritis" OR "complement-mediated membranoproliferative glomerulonephritis" OR "nephropathy due to CFHR5 deficiency"
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: 10 interventional · 0 observational · 1 expanded access. Only interventional studies enter the trial headline.
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 (1797) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity
Ingested 2026-07-27T13:57:24.339Z
