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ORPHA:34145

Immunoglobulin A nephropathy

low confidenceDisorder

Also known as: Berger disease · IgA nephropathy

Publications

27,037

Trials

207

Interventional, condition-specific

Researchers

1,058

Distinct authors in sample

Gene link

—

Readiness

5/6

Stages with a signal

Clinical definition (Orphanet)

A rare glomerular disease, histologically characterized by glomerular mesangial deposits of IgA, often accompanied by IgG and complement C3 as well as mesangioproliferative changes, clinically mostly manifesting as oligosymptomatic glomerulonephritis, possibly infection-triggered macrohematuria and a variable course ranging from spontaneous remission to slow or rarely rapid progression to kidney failure.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (3)

Berger's disease · IgA Nephropathy · IgA 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.”

5/6 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

    27,037 matched papers (16,351 in last 10 years) Source

  3. Phenotype characterisedPresent

    17 HPO annotations (e.g. Foamy urine; Glomerulonephritis; Crackles) Source

  4. Animal modelPresent

    14 genotype models (Mus musculus) Source

  5. Orphan designationPresent

    2 FDA designations (2 FDA orphan-indication approvals) — e.g. blisibimod Source

  6. Interventional trialPresent

    207 matched on ClinicalTrials.gov (72 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)

17

Associated phenotypes · MONDO:0005342

  • Foamy urine
  • Glomerulonephritis
  • Crackles
  • Mild proteinuria
  • Facial edema

Showing 5 of 17 — open Monarch for the full list.

Animal models (Monarch / Alliance)

14

Model associations linked to this Mondo ID

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)

2

Designations · 2 with FDA orphan-indication approval

  • FDA blisibimodImmunoglobulin A Nephropathy · 2017-08-07 · Not FDA Approved for Orphan Indication
  • FDA budesonideImmunoglobulin A Nephropathy Kidney Failure · 2010-05-17 · Not FDA Approved for Orphan Indication

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

64

Drugs / clinical candidates · MONDO_0005342

CTD chemicals (MyDisease.info)

17 associated chemicals · 282 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Adrenal Cortex Hormones · therapeutic
  • Atrasentan · therapeutic
  • Azathioprine · therapeutic
  • Enalapril · therapeutic
  • hydroquinidine · therapeutic
  • Irbesartan · therapeutic
  • Lisinopril · therapeutic
  • Losartan · therapeutic
  • Prednisolone · therapeutic
  • Prednisone · therapeutic
  • Resveratrol · therapeutic
  • sparsentan · therapeutic

Pathways: Other glycan degradation; Glycosaminoglycan degradation; Metabolic pathways; Endocrine resistance; MAPK signaling pathway; cAMP signaling pathway; Cytokine-cytokine receptor interaction; Chemokine signaling pathway

MyDisease.info · MONDO:0005342

Literature

Is anyone studying this?

27,037

27,037 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=3967) is 59.

27,037 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).

16,351 in the last 10 years · low confidence

Phrase hits: 27,037 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,058

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
    Zhang Y12 papers · 2026

    Department of Nephrology, The Second Affiliated Hospital of Nanchang University, Nanchang, Jiangxi, 330006, People's Republic of China.

    Papers in Europe PMC
  2. 02
    Li Y9 papers · 2026

    Department of Health Management Center, The First Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, China.

    Papers in Europe PMC
  3. 03
    Xu G8 papers · 2026

    Department of Nephrology, The Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, No. 1, Minde Road, Donghu District, Nanchang, 330006, P. R. China. gaosixu@163.com.

    Papers in Europe PMC
  4. 04
    Yang Y8 papers · 2026

    Department of Nephrology, The Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, No. 1, Minde Road, Donghu District, Nanchang, 330006, P. R. China.

    Papers in Europe PMC
  5. 05
    Suzuki Y7 papers · 2026

    Department of Nephrology, Juntendo University Faculty of Medicine, Tokyo, Japan.

    Papers in Europe PMC
  6. 06
    Wang Y7 papers · 2026

    Department of Nephrology, The Second Affiliated Hospital, Jiangxi Medical College, Nanchang University, Nanchang, China.

    Papers in Europe PMC
  7. 07
    Chen J6 papers · 2026

    Kidney Disease Center, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.

    Papers in Europe PMC
  8. 08
    Chen X6 papers · 2026

    Department of Nephrology, Fujian Provincial Hospital, Fuzhou University Affiliated Provincial Hospital, Shengli Clinical Medical College of Fujian Medical University, Fuzhou, Fujian, China.

    Papers in Europe PMC
  9. 09
    Li X6 papers · 2026

    School of Public Health, Zhengzhou University, Zhengzhou, 450001, People's Republic of China.

    Papers in Europe PMC
  10. 10
    Wang X6 papers · 2026

    Department of Nephrology, Luwan Branch of Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, ,

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

207

interventional trials for this specific condition

207 interventional trials matched this specific condition name; 72 currently recruiting in our sample.

Data as of 11 September 2026

207 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 99.3th percentile).

low confidence · 99.3th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

35 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 55 · after dedupe 55 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 55 · 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 (55)

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 A nephropathy — 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 A nephropathy" OR "Berger disease" OR "IgA nephropathy" OR "Berger's disease" OR "IgA glomerulonephritis"

Run this search on Europe PMC

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

"Immunoglobulin A nephropathy" OR "Berger disease" OR "IgA nephropathy" OR "Berger's disease" OR "IgA 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: 207 interventional · 35 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 (27037) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-26T23:39:06.749Z