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ORPHA:900

Granulomatosis with polyangiitis

medium confidenceDisorder

Also known as: GPA · Wegener granulomatosis

Publications

23,115

98.7th percentile

Trials

75

Interventional, condition-specific

Researchers

1,135

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis characterized by necrotizing inflammation of small and medium vessels (capillaries, venules and arterioles), resulting in tissue ischemia.

How rare: 1-9 / 100 000 — about one to nine people per hundred thousand.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (6)

Wegener's granulomatosis · Wegener's syndrome · granulomatosis - Wegener's · granulomatosis with polyangiitis · necrotizing respiratory granulomatosis · pauci-immune glomerulonephritis associated with granulomatosis with polyangiitis

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.”

2/6 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

    23,115 matched papers (11,782 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

    75 matched on ClinicalTrials.gov (13 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)

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?

23,115

23,115 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=5449) is 41.

23,115 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).

11,782 in the last 10 years · medium confidence · 98.7th percentile (publications denominator)

Phrase hits: 23,115 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,135

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
    Emmi G4 papers · 2026

    Department of Medical, Surgery and Health Sciences, University of Trieste, Trieste, Italy.

    Papers in Europe PMC
  2. 02
    Avula S3 papers · 2026

    Internal Medicine, University of Minnesota, Minneapolis, USA

    Papers in Europe PMC
  3. 03
    Chen H3 papers · 2026

    Department of Pathology, Hospital for Skin Diseases, Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, China. ch76ch@163.com.

    Papers in Europe PMC
  4. 04
    Higuchi T3 papers · 2026

    Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Tokyo, Japan.

    Papers in Europe PMC
  5. 05
    Mohammad AJ3 papers · 2026

    Section of Rheumatology, Department of Clinical Sciences, Lund University, Lund, Sweden.

    Papers in Europe PMC
  6. 06
    Pagnoux C3 papers · 2026

    Vasculitis Clinic, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada.

    Papers in Europe PMC
  7. 07
    Ueki S3 papers · 2026

    Department of General Internal Medicine and Clinical Laboratory Medicine, Akita University Graduate School of Medicine, Japan.

    Papers in Europe PMC
  8. 08
    Zhou Y3 papers · 2026

    Department of Rheumatology, University of Wisconsin Health, Madison.

    Papers in Europe PMC
  9. 09
    Albayrak F2 papers · 2026

    Department of Rheumatology, Gaziantep University, Gaziantep, Turkey.

    Papers in Europe PMC
  10. 10
    Antiochos B2 papers · 2026

    Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

75

interventional trials for this specific condition

75 interventional trials matched this specific condition name; 13 currently recruiting in our sample.

Data as of 27 July 2026

75 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 98th percentile).

medium confidence · 98th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Observational and natural-history studies

42 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

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.

"Granulomatosis with polyangiitis" OR "Wegener granulomatosis" OR "Wegener's granulomatosis" OR "Wegener's syndrome" OR "granulomatosis - Wegener's" OR "necrotizing respiratory granulomatosis" OR "pauci-immune glomerulonephritis associated with granulomatosis with polyangiitis"

Run this search on Europe PMC

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

"Granulomatosis with polyangiitis" OR "Wegener granulomatosis" OR "Wegener's granulomatosis" OR "Wegener's syndrome" OR "granulomatosis - Wegener's" OR "necrotizing respiratory granulomatosis" OR "pauci-immune glomerulonephritis associated with granulomatosis with polyangiitis"

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: 75 interventional · 42 observational · 0 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: GPA

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 2026-07-26T15:50:10.538Z