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ORPHA:140989

Primary angiitis of the central nervous system

low confidenceDisorder

Also known as: Isolated angiitis of the central nervous system · PACNS · PCNSV · Primary central nervous system vasculitis · Primary vasculitis of the central nervous system

Query health: suspect — Only one of 2 strategies returned hits (phrase).

Publications

1,551

Trials

2

Interventional, condition-specific

Researchers

1,081

Distinct authors in sample

Gene link

—

Readiness

3/6

Stages with a signal

Clinical definition (Orphanet)

A rare, medium or small vessel vasculitis characterized by focal and/or diffuse neurologic symptoms due to a documented arteritic process in the central nervous system, in the absence of other identified underlying cause (infectious, systemic, other neurologic diseases, etc.). It presents with non-specific symptoms of headache, stroke or transient ischemic attacks with cognitive impairment, hemiplegia, weakness, and rarely, with cranial nerve involvement, and .

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

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

Additional Mondo synonyms (4)

isolated angiitis of the central nervous system · primary angiitis of the central nervous system · primary central nervous system vasculitis · primary vasculitis of the central nervous system

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.”

3/6 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

    1,551 matched papers (950 in last 10 years) Source

  3. Phenotype characterisedPresent

    30 HPO annotations (e.g. Hemiparesis; Transient ischemic attack; Cognitive impairment) Source

  4. Animal modelNot found

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

  5. Orphan designationNot found

    No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source

  6. Interventional trialPresent

    2 matched on ClinicalTrials.gov

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)

30

Associated phenotypes · MONDO:0015374

  • Hemiparesis
  • Transient ischemic attack
  • Cognitive impairment
  • Paraparesis
  • Reduced visual acuity

Showing 5 of 30 — 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

No drugs or clinical candidates returned for this Mondo ID on Open Targets.

CTD chemicals (MyDisease.info)

No CTD chemical associations returned for this Mondo ID.

Literature

Is anyone studying this?

1,551

1,551 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.

1,551 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).

950 in the last 10 years · low confidence

Phrase hits: 1,551 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,081

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
    Salvarani C8 papers · 2026

    Department of Neurology, Mayo Clinic, Rochester, MN, USA.

    Papers in Europe PMC
  2. 02
    Zhang X8 papers · 2026

    National Clinical Research Center for Chinese Medicine Acupuncture and Moxibustion, Tianjin, China.

    Papers in Europe PMC
  3. 03
    de Boysson H6 papers · 2026

    Service de Médecine Interne, CHU de Caen, Avenue de la Côte de Nacre, Caen, France; Université Caen Normandie, Caen, France.

    Papers in Europe PMC
  4. 04
    Giannini C6 papers · 2026

    Department of Anatomic Pathology, Mayo Clinic, Rochester, MN, USA.

    Papers in Europe PMC
  5. 05
    Boulouis G5 papers · 2026

    Neuroradiology - Diagnostic and Interventional Neuroradiology, CIC-IT 1415, INSERM 1253 iBrain, Tours University Hospital, Centre Val de Loire Region, France.

    Papers in Europe PMC
  6. 06
    Brown RD Jr5 papers · 2026

    Department of Neurology, Mayo Clinic, Rochester, MN, USA.

    Papers in Europe PMC
  7. 07
    Hunder GG5 papers · 2026

    Division of Rheumatology, Mayo Clinic, Rochester, MN, USA.

    Papers in Europe PMC
  8. 08
    Huston J 3rd5 papers · 2026

    Department of Radiology, Mayo Clinic, Rochester, MN, USA.

    Papers in Europe PMC
  9. 09
    Pagnoux C5 papers · 2026

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

    Papers in Europe PMC
  10. 10
    Pascarella R5 papers · 2025

    Neuroradiology Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

2

interventional trials for this specific condition

2 interventional trials matched this specific condition name; none in our sample are currently recruiting.

Data as of 11 September 2026

2 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 84.5th percentile).

low confidence · 84.5th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

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

No currently recruiting studies in the matched set. Open the same 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 1 · after dedupe 1 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 1 · 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 (1)

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 Primary angiitis of the central nervous system — 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.

"Primary angiitis of the central nervous system" OR "Primary angiitis of central nervous system" OR "Isolated angiitis of the central nervous system" OR "Isolated angiitis of central nervous system" OR "PACNS" OR "PCNSV" OR "Primary central nervous system vasculitis" OR "Primary vasculitis of the central nervous system" OR "Primary vasculitis of central nervous system"

Run this search on Europe PMC

MeSH descriptor terms unioned into the query: Granulomatous Angiitis of the Central Nervous System

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

"Primary angiitis of the central nervous system" OR "Primary angiitis of central nervous system" OR "Isolated angiitis of the central nervous system" OR "Isolated angiitis of central nervous system" OR "PACNS" OR "PCNSV" OR "Primary central nervous system vasculitis" OR "Primary vasculitis of the central nervous system" OR "Primary vasculitis of central nervous system" OR "Granulomatous Angiitis of the Central Nervous System"

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

Query health: suspect — strategies attempted: phrase, mesh; 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 (1551) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity

Ingested 2026-07-27T07:48:56.826Z