Anti-neutrophil cytoplasmic antibody-associated vasculitis
diseaseOn this page
Also known as AAVANCA-associated vasculitisantineutrophil cytoplasmic antibody-associated vasculitis
Summary
Anti-neutrophil cytoplasmic antibody-associated vasculitis (MONDO:0015492) is a disease and 116 clinical trials. Top therapeutic interventions include rituximab, avacopan, and azathioprine. A subtype of necrotizing vasculitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-5 / 10 000 (United States) [Orphanet-validated]
- Clinical trials: 116
Clinical features
Epidemiology
Prevalence records
4 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 3.3 | United States | Validated |
| Point prevalence | 1-5 / 10 000 | 42.1 | United States | Validated |
| Point prevalence | 1-5 / 10 000 | Europe | Not yet validated | |
| Point prevalence | 1-5 / 10 000 | 14.9 | Germany | Not yet validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | anti-neutrophil cytoplasmic antibody-associated vasculitis |
| Mondo ID | MONDO:0015492 |
| MeSH | D056648 |
| Orphanet | 156152 |
| ICD-11 | 1404622826 |
| UMLS | C2717865 |
| MedGen | 403453 |
| GARD | 0013011 |
| Is cancer (heuristic) | no |
Also known as: AAV · ANCA-associated vasculitis · antineutrophil cytoplasmic antibody-associated vasculitis
Disease family
This is a subtype of necrotizing vasculitis. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › cardiovascular disorder › vascular disorder › vasculitis › necrotizing vasculitis › anti-neutrophil cytoplasmic antibody-associated vasculitis
Related subtypes (2): immunoglobulin A vasculitis, disseminated visceral giant cell angiitis
Subtypes (2): granulomatosis with polyangiitis, microscopic polyangiitis
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
Drugs indicated for this disease
1 approved. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Avacopan | Approved (phase 4) |
Clinical trials & evidence
Clinical trials
Clinical trials: 116.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 41 |
| PHASE1 | 22 |
| PHASE2 | 13 |
| EARLY_PHASE1 | 12 |
| PHASE4 | 10 |
| PHASE3 | 9 |
| PHASE1/PHASE2 | 9 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT05962840 | PHASE4 | RECRUITING | Efficacy and Safety for Rituximab Combined With Telitacicept in the Treatment of ANCA-associated Vasculitis (TTCAAVREM) |
| NCT05965284 | PHASE4 | RECRUITING | Efficacy and Safety for Telitacicept in the Remission Maintenance Treatment of ANCA-associated Vasculitis (TTCAZAREM) |
| NCT06072482 | PHASE4 | RECRUITING | A Study to Evaluate Avacopan in Participants With ANCA-associated Vasculitis |
| NCT06611696 | PHASE4 | RECRUITING | Avacopan vs Reduced-dose Glucocorticoids in ANCA-associated Vasculitis |
| NCT07035834 | PHASE4 | NOT_YET_RECRUITING | SGLT-2 Inhibitors on Albuminuria in Chronic Kidney Disease Patients With Lupus Nephritis and ANCA- Associated Vasculitis |
| NCT07451847 | PHASE4 | NOT_YET_RECRUITING | Comparison of a Strategy Based on Clinico-biological Monitoring Versus Pre-emptive Rituximab Treatment in Cases of ANCA Reappearance in Granulomatosis With Polyangiitis and Microscopic Polyangiitis. |
| NCT02198248 | PHASE4 | UNKNOWN | Low-dose Glucocorticoid Vasculitis Induction Study |
| NCT02749292 | PHASE4 | TERMINATED | Maintenance of ANCA Vasculitis Remission by Intermittent Rituximab Dosing |
| NCT04316494 | PHASE4 | TERMINATED | Hydroxychloroquine in ANCA Vasculitis Evaluation |
| NCT05969522 | PHASE4 | UNKNOWN | Stratified Therapy on Pediatric AAGN |
| NCT03323476 | PHASE3 | ACTIVE_NOT_RECRUITING | Maintaining or Stopping Immunosuppressive Therapy in Patients With ANCA Vasculitis and End-stage Renal Disease |
| NCT04871191 | PHASE3 | RECRUITING | Study of Salvage Therapy to Treat Patients With Granulomatosis With Polyangiitis |
| NCT05946564 | PHASE3 | RECRUITING | A Trial to Evaluate the Efficacy of Pioglitazone to Promote Renal Tolerance in ANCA-associated Vasculitis - RENATO Trial |
| NCT07168161 | PHASE3 | RECRUITING | BDB-001 Phase III Trial in ANCA-Associated Vasculitis |
| NCT07373262 | PHASE3 | NOT_YET_RECRUITING | Avacopan Added to Standard-of-care Therapy in ANCA-associated Vasculitis With Severe Kidney Involvement |
| NCT01697267 | PHASE3 | COMPLETED | Rituximab Vasculitis Maintenance Study |
| NCT02108860 | PHASE3 | COMPLETED | Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener’s) |
| NCT02994927 | PHASE3 | COMPLETED | A Phase 3 Clinical Trial of CCX168 (Avacopan) in Patients With ANCA-Associated Vasculitis |
| NCT03942887 | PHASE3 | UNKNOWN | Exploring Durable Remission With Rituximab in Antineutrophil Cytoplasmic Antibody(ANCA)-Associated Vasculitis |
| NCT05630612 | PHASE2 | ACTIVE_NOT_RECRUITING | ETA and AT1 Antagonism in ANCA-vasculitis (SPARVASC) |
| NCT05732402 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | An Open-label Study of Povetacicept in Autoantibody-Associated Glomerular Diseases |
| NCT06226662 | PHASE2 | NOT_YET_RECRUITING | Study of NM8074 in Patients with Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) |
| NCT06350110 | PHASE1/PHASE2 | RECRUITING | Fourth-gen CAR T Cells Targeting BCMA/CD19 for Refractory Systemic Lupus Erythematosus (SLE) |
| NCT06685042 | PHASE1/PHASE2 | RECRUITING | Anti-CD19 CAR T-Cell Therapy in Refractory Systemic Autoimmune Diseases |
| NCT06733935 | PHASE1/PHASE2 | RECRUITING | A Phase 1/2 Study of NKX019 in Subjects With Immune-Mediated Diseases (Ntrust-2) |
| NCT06794008 | PHASE2 | RECRUITING | BCMA-CD19 CAR-T Therapy for Refractory Autoimmune Diseases |
| NCT06821659 | PHASE1/PHASE2 | NOT_YET_RECRUITING | Safety and Efficacy of Universal CAR-T Cells (UWD-CD19) Combined with Immunosuppressants in the Treatment of Refractory Autoimmune Diseases |
| NCT06828042 | PHASE1/PHASE2 | RECRUITING | Safety and Efficacy of Universal CD19-targeting CAR-γδT Cells in Refractory Autoimmune Diseases |
| NCT06868290 | PHASE2 | RECRUITING | Phase 2 Study Evaluating Rapcabtagene Autoleucel in Participants With Severe Active GPA or MPA |
| NCT07089121 | PHASE1/PHASE2 | RECRUITING | Descartes-08 for Children, Adolescents, and Young Adults With Autoimmune Disorders |
| NCT07160608 | PHASE2 | RECRUITING | Safety and Efficacy of Tarperprumig in Adult Participants With Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis |
| NCT07388277 | PHASE1/PHASE2 | RECRUITING | CC-97540 in Patients With Antineutrophil Cytoplasmic Antibody-associated Vasculitis |
| NCT00293072 | PHASE2 | COMPLETED | Pilot Study of Rituximab Therapy for Systemic Lupus Erythematosus (SLE) and Vasculitis |
| NCT00482066 | PHASE2 | TERMINATED | Abatacept in ANCA Associated Vasculitis |
| NCT01275274 | PHASE2 | WITHDRAWN | Retinoids in ANCA Small Vessel Vasculitis: Silencing Autoantigens |
| NCT01275287 | PHASE2 | WITHDRAWN | Targeting Complement Activation in Antineutrophil Cytoplasmic Autoantibodies (ANCA)-Vasculitis - Eculizumab |
| NCT01633476 | PHASE2 | UNKNOWN | CMV Modulation of the Immune System in ANCA-associated Vasculitis |
| NCT02222155 | PHASE2 | COMPLETED | Clinical Trial to Evaluate Safety and Efficacy of CCX168 in ANCA-Associated Vasculitis |
| NCT03967925 | PHASE2 | UNKNOWN | Rituximab and Belimumab Combination Therapy in PR3 Vasculitis |
| NCT05197842 | PHASE1/PHASE2 | COMPLETED | Efficacy and Safety of Substitution of Glucocorticoid for BDB-001 Injection in Patients With Anti-neutrophil Cytoplasmic Antibody(ANCA)-Associated Vasculitis |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| RITUXIMAB | 4 | 12 |
| AVACOPAN | 4 | 7 |
| AZATHIOPRINE | 4 | 4 |
| TOFACITINIB | 4 | 3 |
| ABATACEPT | 4 | 2 |
| BELIMUMAB | 4 | 1 |
| ECULIZUMAB | 4 | 1 |
| HYDROXYCHLOROQUINE | 4 | 1 |
| LEFLUNOMIDE | 4 | 1 |
| SPARSENTAN | 4 | 1 |
| TRETINOIN | 4 | 1 |
| VALACYCLOVIR | 4 | 1 |
| TELITACICEPT | 3 | 2 |
| LORNOXICAM | 3 | 1 |
| GREZIPRUBART | 2 | 1 |
| POVETACICEPT | 2 | 1 |
| RAPCABTAGENE AUTOLEUCEL | 2 | 1 |
| RUXOPRUBART | 2 | 1 |
| TARPERPRUMIG | 2 | 1 |
| ZOLACABTAGENE AUTOLEUCEL | 1 | 1 |
| CHEMBL1235644 | 0 | 1 |
| CHEMBL4877807 | 0 | 1 |