Kisho

Kisho is an information platform, not a medical provider. Nothing on this site constitutes medical advice, diagnosis, or treatment recommendations. All content is aggregated from publicly available sources (including ClinicalTrials.gov, PubMed, FDA.gov, and Orphanet) and is provided for informational purposes only. Clinical trial eligibility, treatment decisions, and any health-related actions should always be discussed with a qualified healthcare professional. Kisho does not endorse any specific therapy, organization, or clinical trial. Terms of use · Privacy policy

anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

anti-neutrophil cytoplasmic antibody-associated vasculitis

MONDO:0015492

Also known as:AAVANCA-associated vasculitisantineutrophil cytoplasmic antibody-associated vasculitis

Find Clinical TrialsTrials

Overview

Group of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.

TreatmentsNo approved treatments · 1 in pipeline

Clinical Trials44 registered · 25 recruiting

Diagnosis

Biomarker and diagnostic research for anti-neutrophil cytoplasmic antibody-associated vasculitis has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for anti-neutrophil cytoplasmic antibody-associated vasculitis. An additional 1 compound holds orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for anti-neutrophil cytoplasmic antibody-associated vasculitis, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for anti-neutrophil cytoplasmic antibody-associated vasculitis. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name

Prognosis

Estimated prevalence: 1-5 in 10,000 (Uncommon).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

44 clinical trials registered, 25 recruiting. Interventions under study include drug therapy, other interventions, and biologic therapy. Pipeline includes 2 PHASE4, 5 PHASE3, 4 PHASE2. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT02257866](https://clinicaltrials.gov/study/NCT02257866)

Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 7:39 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:156152

GARD

Genetic and Rare Diseases Info Center

GARD:13011

Questions for Your Care Team

→Are any of the 44 active clinical trials a good fit for my situation?
→What is the current development status of Gabexate, and when might it become available?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Research summaries	85	43%
Disease patterns and progression	49	25%
Laboratory research	20	10%
Patient case studies	16	8%
Testing and diagnosis research	14	7%
Clinical study results	9	5%
New treatment approaches	4	2%
Other research	3	2%

Kisho

anti-neutrophil cytoplasmic antibody-associated vasculitis

Overview

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications

Latest News & Developments

Anti-neutrophil cytoplasmic antibody-associated vasculitis presenting with acute pancreatitis, jaundice, severe anaemia and upper gastrointestinal bleed.

Concurrent anti-neutrophil cytoplasmic antibody-associated nephritis and IgG4-related tubulointerstitial nephritis: a rare case.

Antineutrophil cytoplasmic antibody-associated pachymeningitis: a systematic review of clinical features, diagnosis and treatment outcomes.

anti-neutrophil cytoplasmic antibody-associated vasculitis

Overview

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

Recent Publications

Latest News & Developments

Anti-neutrophil cytoplasmic antibody-associated vasculitis presenting with acute pancreatitis, jaundice, severe anaemia and upper gastrointestinal bleed.

Concurrent anti-neutrophil cytoplasmic antibody-associated nephritis and IgG4-related tubulointerstitial nephritis: a rare case.

Antineutrophil cytoplasmic antibody-associated pachymeningitis: a systematic review of clinical features, diagnosis and treatment outcomes.