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ORPHA:183

Eosinophilic granulomatosis with polyangiitis

medium confidenceDisorder

Also known as: Churg-Strauss syndrome · EGPA · Granulomatous allergic angiitis

Publications

10,278

95.2th percentile

Trials

30

Interventional, condition-specific

Researchers

1,169

Distinct authors in sample

Gene link

—

Readiness

4/6

Stages with a signal

Clinical definition (Orphanet)

A rare systemic vasculitis of small vessels characterized by asthma, blood and tissue eosinophilia and vasculitis manifestations.

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 (35)

CSS · Churg Strauss Syndrome · Churg Strauss syndrome · Churg-Strauss vasculitis · allergic angiitides · allergic angiitides, granulomatous · allergic angiitis · allergic angiitis and granulomatosis · allergic angiitis, granulomatous · allergic granulomatoses · allergic granulomatosis · allergic granulomatous Angiitides · allergic granulomatous and angiitis · allergic granulomatous angiitis · angiitides, allergic · angiitides, allergic granulomatous · angiitides, granulomatous allergic · angiitis, allergic · angiitis, allergic granulomatous · angiitis, granulomatous allergic · eosinophilic granulomatous Vasculitides · eosinophilic granulomatous vasculitis · granulomatoses, allergic · granulomatosis, allergic · granulomatous allergic Angiitides · granulomatous allergic angiitis · granulomatous angiitides, allergic · granulomatous angiitis, allergic · granulomatous vasculitides, eosinophilic · granulomatous vasculitis, eosinophilic · syndrome, Churg-Strauss · vasculitides, eosinophilic granulomatous · vasculitis, Churg Strauss · vasculitis, Churg-Strauss · vasculitis, eosinophilic granulomatous

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

    10,278 matched papers (5,841 in last 10 years) Source

  3. Phenotype characterisedPresent

    68 HPO annotations (e.g. Asthma; Central nervous system degeneration; Hypertension) Source

  4. Animal modelNot found

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

  5. Orphan designationPresent

    4 FDA · 2 EMA designations (2 FDA orphan-indication approvals) — e.g. benralizumab Source

  6. Interventional trialPresent

    30 matched on ClinicalTrials.gov (6 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)

68

Associated phenotypes · MONDO:0015943

  • Asthma
  • Central nervous system degeneration
  • Hypertension
  • Gait disturbance
  • Abnormal pleura morphology

Showing 5 of 68 — 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 · 2 with FDA orphan-indication approval

  • FDA benralizumabEosinophilic granulomatosis with polyangiitis · 2018-11-21 · Not FDA Approved for Orphan Indication
  • FDA avacopanChurg-Strauss Syndrome Vasculitides Microscopic Polyangiitis Granulomatosis with polyangiitis Wegener Granulomatosis · 2014-06-02 · Not FDA Approved for Orphan Indication
  • EMA Methyl-(1-{[6-{[(1S)-1-cyclopropylethyl]amino}-2-(pyrazolo[5,1-b][1,3]thiazol-7-yl)-pyrimidin-4-yl]carbonyl}piperidin-4-yl)carbamate mono(4-methylbenzenesulfonate)Treatment of eosinophilic granulomatosis with polyangiitis · 12/01/2024 · PositiveEMA designation
  • FDA mepolizumab (NUCALA)Churg-Strauss Syndrome · 2011-07-14
  • FDA rituximab (Rituxan)Churg-Strauss Syndrome Microscopic Polyangiitis Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Wegener's Granulomatosis · 2006-02-14
  • EMA MepolizumabTreatment of Churg-Strauss syndrome · 12/03/2013 · WithdrawnEMA designation

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

19

Drugs / clinical candidates · MONDO_0015943

CTD chemicals (MyDisease.info)

19 associated chemicals. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Adrenal Cortex Hormones · therapeutic
  • Azathioprine · therapeutic
  • Cortisone · therapeutic
  • Cyclophosphamide · therapeutic
  • Methylprednisolone · therapeutic
  • Prednisolone · therapeutic
  • Prednisone · therapeutic
  • Steroids · therapeutic
  • Acetaminophen · marker/mechanism
  • Azithromycin · marker/mechanism
  • Carbamazepine · marker/mechanism
  • Carbimazole · marker/mechanism

MyDisease.info · MONDO:0015943

Literature

Is anyone studying this?

10,278

10,278 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.

10,278 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).

5,841 in the last 10 years · medium confidence · 95.2th percentile (publications denominator)

Phrase hits: 10,278 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,169

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
    Wechsler ME10 papers · 2026

    Department of Medicine, National Jewish Health, Denver, Colorado.

    Papers in Europe PMC
  2. 02
    Hellmich B8 papers · 2026

    Klinik für Innere Medizin, Rheumatologie, Pneumologie, Nephrologie und Diabetologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany.

    Papers in Europe PMC
  3. 03
    Emmi G7 papers · 2026

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

    Papers in Europe PMC
  4. 04
    Terrier B7 papers · 2026

    Cochin Hospital, Paris, France.

    Papers in Europe PMC
  5. 05
    Vaglio A7 papers · 2026

    Department of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Florence, Florence, Italy.

    Papers in Europe PMC
  6. 06
    Higuchi T5 papers · 2026

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

    Papers in Europe PMC
  7. 07
    Merkel PA5 papers · 2026

    Division of Rheumatology, Department of Medicine, Division of Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, Pennsylvania.

    Papers in Europe PMC
  8. 08
    Pagnoux C5 papers · 2026

    Mount Sinai Hospital, University Health Network, Toronto, ON, Canada.

    Papers in Europe PMC
  9. 09
    Tamura N5 papers · 2026

    Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Japan.

    Papers in Europe PMC
  10. 10
    Tanaka K5 papers · 2026

    Nephrology and Rheumatology center, and Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Tokyo, 105-8470, Japan.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

30

interventional trials for this specific condition

30 interventional trials matched this specific condition name; 6 currently recruiting in our sample. 25 trials are registered for granulomatosis with polyangiitis, the broader category — shown separately because they may or may not enrol this specific subtype.

Data as of 11 September 2026

30 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 96th percentile).

medium confidence · 96th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

Broader category: granulomatosis with polyangiitis

25

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

Observational and natural-history studies

26 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 13 · after dedupe 13 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 13 · 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 (13)

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 Eosinophilic granulomatosis with polyangiitis — 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.

("Eosinophilic granulomatosis with polyangiitis" OR "Churg-Strauss syndrome" OR "Granulomatous allergic angiitis" OR "Churg Strauss Syndrome" OR "Churg-Strauss vasculitis" OR "allergic angiitides" OR "allergic angiitides, granulomatous" OR "allergic angiitis" OR "allergic angiitis and granulomatosis" OR "allergic angiitis, granulomatous" OR "allergic granulomatoses" OR "allergic granulomatosis" OR "allergic granulomatous Angiitides" OR "allergic granulomatous and angiitis" OR "allergic granulomatous angiitis" OR "angiitides, allergic" OR "angiitides, allergic granulomatous" OR "angiitides, granulomatous allergic" OR "angiitis, allergic" OR "angiitis, allergic granulomatous" OR "angiitis, granulomatous allergic" OR "eosinophilic granulomatous Vasculitides" OR "eosinophilic granulomatous vasculitis" OR "granulomatoses, allergic" OR "granulomatosis, allergic" OR "granulomatous allergic Angiitides" OR "granulomatous angiitides, allergic" OR "granulomatous angiitis, allergic" OR "granulomatous vasculitides, eosinophilic" OR "granulomatous vasculitis, eosinophilic" OR "syndrome, Churg-Strauss" OR "vasculitides, eosinophilic granulomatous" OR "vasculitis, Churg Strauss" OR "vasculitis, Churg-Strauss" OR "vasculitis, eosinophilic granulomatous")

Run this search on Europe PMC

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

"Eosinophilic granulomatosis with polyangiitis" OR "Churg-Strauss syndrome" OR "Granulomatous allergic angiitis" OR "Churg Strauss Syndrome" OR "Churg-Strauss vasculitis" OR "allergic angiitides" OR "allergic angiitides, granulomatous" OR "allergic angiitis" OR "allergic angiitis and granulomatosis" OR "allergic angiitis, granulomatous" OR "allergic granulomatoses" OR "allergic granulomatosis" OR "allergic granulomatous Angiitides" OR "allergic granulomatous and angiitis" OR "allergic granulomatous angiitis" OR "angiitides, allergic" OR "angiitides, allergic granulomatous" OR "angiitides, granulomatous allergic" OR "angiitis, allergic" OR "angiitis, allergic granulomatous" OR "angiitis, granulomatous allergic" OR "eosinophilic granulomatous Vasculitides" OR "eosinophilic granulomatous vasculitis" OR "granulomatoses, allergic" OR "granulomatosis, allergic" OR "granulomatous allergic Angiitides" OR "granulomatous angiitides, allergic" OR "granulomatous angiitis, allergic" OR "granulomatous vasculitides, eosinophilic" OR "granulomatous vasculitis, eosinophilic" OR "syndrome, Churg-Strauss" OR "vasculitides, eosinophilic granulomatous" OR "vasculitis, Churg Strauss" OR "vasculitis, Churg-Strauss" OR "vasculitis, eosinophilic granulomatous"

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: 30 interventional · 26 observational · 0 expanded access. Only interventional studies enter the trial headline.

Parent-category trials query:

"granulomatosis with polyangiitis"

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

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: EGPA; CSS

Confidence reasoning

  • Preferred label is multi-word and distinctive
  • 2 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T12:48:06.321Z