Vasculitis

Summary

Vasculitis (MONDO:0018882) is a disease (an umbrella term covering 18 Mondo subtypes) with 5 cohort genes (19 GWAS associations across 3 studies) and 98 clinical trials. Top therapeutic interventions include azathioprine, avacopan, and cyclophosphamide anhydrous.

At a glance

• Prevalence: 1-9 / 100 000 (Europe)
• Umbrella term: 18 Mondo subtypes
• Cohort genes: 5
• GWAS associations: 19
• ClinVar variants: 2
• Clinical trials: 98

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	1-9 / 100 000	6.3	Europe	Not yet validated

Identifiers

Disease identifiers

Field	Value
Canonical name	vasculitis
Mondo ID	MONDO:0018882
EFO	EFO:0006803
MeSH	D014657
Orphanet	52759
DOID	DOID:865
ICD-11	572581721
NCIT	C26912
SNOMED CT	31996006
UMLS	C0042384
MedGen	12054
GARD	0018844
MedDRA	10036023, 10047115
Is cancer (heuristic)	no

Also known as: systemic vasculitis

Data availability: 2 ClinVar variants · 19 GWAS associations (3 studies).

Disease family

An umbrella term covering 18 Mondo subtypes.

Classification path: disease › human disease › disease by body system or component › cardiovascular disorder › vascular disorder › vasculitis

Related subtypes (59): arterial disorder, ischemic colitis, thrombotic disease, capillary disorder, angiodysplasia, hepatic vascular disorder, vascular hemostatic disease, vein disorder, ischemic disease, peripheral vascular disease, venous thromboembolism, ocular vascular disorder, cholesterol embolism, thoracic outlet syndrome, idiopathic spontaneous coronary artery dissection, cerebral arteriopathy with subcortical infarcts and leukoencephalopathy, angioosteohypertrophic syndrome, Bannayan-Riley-Ruvalcaba syndrome, arterial tortuosity syndrome, hereditary arterial and articular multiple calcification syndrome, pulmonary venoocclusive disease, multiple cutaneous and mucosal venous malformations, arterial dissection-lentiginosis syndrome, patent ductus arteriosus, multisystemic smooth muscle dysfunction syndrome, STING-associated vasculopathy with onset in infancy, capillary malformation, Ehlers-Danlos syndrome, vascular-like type, calciphylaxis, neonatal Marfan syndrome, Ehlers-Danlos syndrome, vascular type, lethal arteriopathy syndrome due to fibulin-4 deficiency, congenital portosystemic shunt, arterial calcification of infancy, Loeys-Dietz syndrome, skin vascular disease, lymphatic malformation, familial thoracic aortic aneurysm and aortic dissection, congenital anomaly of superior vena cava, congenital anomaly of the inferior vena cava, congenital anomaly of hepatic vein, congenital renal artery stenosis, internal carotid agenesis, coronary sinus stenosis, coronary sinus atresia, vascular occlusion disorder, vascular insufficiency disorder, blood vessel neoplasm, vascular ectasia, vascular disorder of penis, fibrocartilaginous embolism, vascular malformation, lymphatic vessel neoplasm, neurovascular disorder, superior vena cava syndrome, coronary microvascular disorder, segmental arterial mediolysis, bleeding disorder, vascular-type, arterial tortuosity-bone fragility syndrome

Subtypes (18): choroiditis, Shwartzman phenomenon, central nervous system vasculitis, phlebitis, lymphangitis, aortitis, hypersensitivity vasculitis, retinal vasculitis, vasculitis, lymphocytic, nodular, Kawasaki disease, deficiency of adenosine deaminase 2, immune complex mediated vasculitis, secondary vasculitis, cutaneous vasculitis, livedoid vasculopathy, autoimmune vasculitis, arteritis, necrotizing vasculitis

Genetics & variants

GWAS landscape

19 GWAS associations across 3 studies. Top hits map to 15 distinct genes (as reported by GWAS).

Top associations by p-value

rsID	p-value	Gene	Risk allele	Odds ratio
rs2242944	1e-10	LINC02940 - RPL23AP12	A	0.75
rs7725339	2e-10	IL12B-AS1	T	1.24
rs11209039	6e-10	DNAJB6P4 - IL12RB2	G	0.81
rs3743841	8e-10	NAGPA	T
rs128738	3e-09	P4HA2	T	1.32
rs4690319	5e-09	CPLX1 - GAK	A	0.82
rs1837253	8e-09	BCLAF1P1 - TSLP	T	0.65
rs6894249	8e-09	CARINH, IRF1	?
rs2087726	1e-08	CCR3	G	0.81
rs73223431	2e-08	PTK2B	T	0.73
rs10995248	2e-08	LINC02929	?
rs62184865	2e-08	RNU6-474P - CTLA4	T	1.72
rs4252120	3e-08	PLG	C	1.28
rs72836352	4e-08	MIR4435-2HG, BCL2L11	T	1.83
rs60689680	5e-08	IL12B-AS1	?
rs73379591	7e-08	EPB41L3	C
rs10508313	1e-07	LINP1	C
rs16925200	6e-07	KDM4C	C	1.75
rs6704162	4e-06	RGS2-AS1	G	1.25

Top studies (by case count)

Study	Lead author	Year	Cases	Controls	Title
GCST012636	Ortiz-Fernandez L	2018	2,425	4,526	Cross-phenotype analysis of Immunochip data identifies KDM4C as a relevant locus for the development of systemic vasculitis.
GCST90095078	Carmona EG	2021	402	7,538	Identification of a shared genetic risk locus for Kawasaki disease and IgA vasculitis by a cross-phenotype meta-analysis.
GCST90271348	Ortiz-Fernandez L	2023	0	0	Identification of new risk loci shared across systemic vasculitides points towards potential target genes for drug repurposing.

Variant details and genetic-evidence tiers

Tier distribution (top 50 variants)

Tier	Variants
Tier 1: coding	0
Tier 2: splice/UTR	0
Tier 3: regulatory	0
Tier 4: intronic/intergenic	19

MAF distribution

Bucket	Variants
common (>=0.05)	19
low_freq (0.01-0.05)	0
rare (<0.01)	0
unknown	0

Functional consequences

Consequence	Count
intron_variant	12
intergenic_variant	7

Top variants

rsID	Chr	Pos	Alleles	MAF	Consequence	Gene	p-value	Tier
rs2242944	21	39093252	G>A,C	0.05	intergenic_variant	LINC02940 - RPL23AP12	1e-10	Tier 4: intronic/intergenic
rs7725339	5	159349993	G>C,T	0.05	intergenic_variant	IL12B-AS1	2e-10	Tier 4: intronic/intergenic
rs11209039	1	67285510	A>G,T	0.05	intergenic_variant	DNAJB6P4 - IL12RB2	6e-10	Tier 4: intronic/intergenic
rs3743841	16	5030085	C>G,T	0.05	intron_variant	NAGPA	8e-10	Tier 4: intronic/intergenic
rs128738	5	132205182	G>A,T	0.05	intron_variant	P4HA2	3e-09	Tier 4: intronic/intergenic
rs4690319	4	831200	A>G,T	0.05	intergenic_variant	CPLX1 - GAK	5e-09	Tier 4: intronic/intergenic
rs1837253	5	111066174	T>C	0.05	intron_variant	BCLAF1P1 - TSLP	8e-09	Tier 4: intronic/intergenic
rs6894249	5	132461855	A>C,G,T	0.05	intron_variant	CARINH, IRF1	8e-09	Tier 4: intronic/intergenic
rs2087726	3	46166818	G>A	0.05	intron_variant	CCR3	1e-08	Tier 4: intronic/intergenic
rs73223431	8	27362470	C>A,T	0.05	intron_variant	PTK2B	2e-08	Tier 4: intronic/intergenic
rs10995248	10	62636282	C>A,G,T	0.05	intron_variant	LINC02929	2e-08	Tier 4: intronic/intergenic
rs62184865	2	203824653	G>T	0.05	intergenic_variant	RNU6-474P - CTLA4	2e-08	Tier 4: intronic/intergenic
rs4252120	6	160722576	T>C,G	0.05	intron_variant	PLG	3e-08	Tier 4: intronic/intergenic
rs72836352	2	111137297	C>T	0.05	intron_variant	MIR4435-2HG, BCL2L11	4e-08	Tier 4: intronic/intergenic
rs60689680	5	159407359	G>A,T	0.05	intergenic_variant	IL12B-AS1	5e-08	Tier 4: intronic/intergenic
rs73379591	18	5503378	T>C	0.05	intron_variant	EPB41L3	7e-08	Tier 4: intronic/intergenic
rs10508313	10	6791131	C>T	0.05	intron_variant	LINP1	1e-07	Tier 4: intronic/intergenic
rs16925200	9	7045058	T>C	0.05	intron_variant	KDM4C	6e-07	Tier 4: intronic/intergenic
rs6704162	1	192445292	A>G	0.05	intergenic_variant	RGS2-AS1	4e-06	Tier 4: intronic/intergenic

ClinVar germline variants

2 retrieved; paginated sample, class counts are floors:

2 uncertain significance

ClinVar	Variant (HGVS)	Gene	Classification	Review
523540	NM_031443.4(CCM2):c.1234C>T (p.Arg412Trp)	CCM2	Uncertain significance	criteria provided, multiple submitters, no conflicts
523445	NM_001846.4(COL4A2):c.1776+13A>G	COL4A2	Uncertain significance	criteria provided, single submitter

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 3 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
CCM2	Orphanet:221061	Familial cerebral cavernous malformation
COL4A2	Orphanet:36383	COL4A1/2-related familial vascular leukoencephalopathy
COL4A2	Orphanet:99810	Familial porencephaly

Cohort genes → proteins

5 cohort genes, 5 distinct canonical proteins.

Evidence partition

Subset	Genes
gwas_only	3
multi_evidence	2

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
KDM4C	HGNC:17071	ENSG00000107077	Q9H3R0	Lysine-specific demethylase 4C	gwas
CCM2	HGNC:21708	ENSG00000136280	Q9BSQ5	Cerebral cavernous malformations 2 protein	clinvar
COL4A2	HGNC:2203	ENSG00000134871	P08572	Collagen alpha-2(IV) chain	clinvar
RGS21	HGNC:26839	ENSG00000253148	Q2M5E4	Regulator of G-protein signaling 21	gwas
RGS1	HGNC:9991	ENSG00000090104	Q08116	Regulator of G-protein signaling 1	gwas

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
KDM4C	Lysine-specific demethylase 4C	Histone demethylase that specifically demethylates ‘Lys-9’ and ‘Lys-36’ residues of histone H3, thereby playing a central role in histone code.
CCM2	Cerebral cavernous malformations 2 protein	Component of the CCM signaling pathway which is a crucial regulator of heart and vessel formation and integrity.
COL4A2	Collagen alpha-2(IV) chain	Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a ‘chicken-wire’ meshwork together with laminins, proteoglycans and entactin/nidogen.
RGS21	Regulator of G-protein signaling 21	Inhibits signal transduction by increasing the GTPase activity of G protein alpha subunits thereby driving them into their inactive GDP-bound form.
RGS1	Regulator of G-protein signaling 1	Regulates G protein-coupled receptor signaling cascades, including signaling downstream of the N-formylpeptide chemoattractant receptors and leukotriene receptors.

Protein-family classification

Druggable: 0 · Difficult: 1 · Unknown: 4 · Druggable fraction: 0.0

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

Family	Genes	Fold	FDR
Transcription factor	1	1.6×	0.476
Other/Unknown	4	1.4×	0.476

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
KDM4C	Transcription factor	no	1.14.11.27	Znf_PHD, Tudor, JmjC_dom
CCM2	Other/Unknown	no		PTB/PI_dom, PH-like_dom_sf, Malcavernin
COL4A2	Other/Unknown	no		Collagen_IV_NC, Collagen, CTDL_fold
RGS21	Other/Unknown	no		RGS, RGS_subdom1/3, RGS_sf
RGS1	Other/Unknown	no		RGS, RGS_subdom1/3, RGS_sf

Expression context

Cohort genes with no expression data: 0.

5 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

Bucket	Genes
narrow (1-5 tissues)	1
moderate (6-20)	0
broad (>20)	4
unknown	0

Top tissues across cohort

Tissue	Cohort genes
cerebellar cortex	1
cerebellar hemisphere	1
right hemisphere of cerebellum	1
anterior cingulate cortex	1
nucleus accumbens	1
putamen	1
decidua	1
placenta	1
saphenous vein	1
colonic epithelium	1
ganglionic eminence	1
sural nerve	1
C1 segment of cervical spinal cord	1
olfactory bulb	1
right lung	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
KDM4C	250	ubiquitous	marker	right hemisphere of cerebellum, cerebellar hemisphere, cerebellar cortex
CCM2	243	ubiquitous	marker	putamen, nucleus accumbens, anterior cingulate cortex
COL4A2	284	ubiquitous	marker	saphenous vein, decidua, placenta
RGS21	1		marker	ganglionic eminence, sural nerve, colonic epithelium
RGS1	260	broad	marker	C1 segment of cervical spinal cord, olfactory bulb, right lung

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
COL4A2	2,746
RGS1	2,264
CCM2	1,600
KDM4C	1,380
RGS21	984

Structural data

PDB: 4 · AlphaFold-only: 1 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
CCM2	Q9BSQ5	8
KDM4C	Q9H3R0	7
COL4A2	P08572	4
RGS1	Q08116	2

AlphaFold-only cohort genes (top 30 by pLDDT)

Symbol	UniProt	pLDDT
RGS21	Q2M5E4	85.05

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 26. Enrichment computed across 5 evidence-associated genes (4 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 4 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Anchoring fibril formation	1	190.3×	0.030	COL4A2
Scavenging by Class A Receptors	1	150.3×	0.030	COL4A2
Fibronectin matrix formation	1	142.8×	0.030	COL4A2
Crosslinking of collagen fibrils	1	142.8×	0.030	COL4A2
Attachment of bacteria to epithelial cells	1	124.1×	0.030	COL4A2
G alpha (q) signalling events	2	28.7×	0.030	RGS21, RGS1
G alpha (i) signalling events	2	19.5×	0.030	RGS21, RGS1
Laminin interactions	1	95.2×	0.034	COL4A2
RHO GTPases activate PKNs	1	79.3×	0.035	KDM4C
Collagen chain trimerization	1	64.9×	0.035	COL4A2
Signaling by PDGF	1	63.4×	0.035	COL4A2
NCAM1 interactions	1	62.1×	0.035	COL4A2
HDMs demethylate histones	1	57.1×	0.035	KDM4C
Assembly of collagen fibrils and other multimeric structures	1	50.1×	0.036	COL4A2
Collagen degradation	1	43.9×	0.036	COL4A2
Collagen biosynthesis and modifying enzymes	1	42.6×	0.036	COL4A2
Activated PKN1 stimulates transcription of AR (androgen receptor) regulated genes KLK2 and KLK3	1	42.0×	0.036	KDM4C
Non-integrin membrane-ECM interactions	1	38.6×	0.036	COL4A2
ECM proteoglycans	1	37.6×	0.036	COL4A2
Integrin cell surface interactions	1	33.6×	0.038	COL4A2
Chromatin organization	1	20.4×	0.060	KDM4C
Chromatin modifying enzymes	1	18.1×	0.064	KDM4C
RHO GTPase Effectors	1	17.0×	0.065	KDM4C
Signaling by Rho GTPases	1	8.6×	0.119	KDM4C
Signaling by Rho GTPases, Miro GTPases and RHOBTB3	1	8.4×	0.119	KDM4C
Signal Transduction	1	2.5×	0.339	KDM4C

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 5 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
negative regulation of signal transduction	2	149.8×	0.003	RGS21, RGS1
leukotriene signaling pathway	1	3370.4×	0.007	RGS1
endothelial cell development	1	842.6×	0.012	CCM2
blood vessel endothelial cell differentiation	1	674.1×	0.012	CCM2
sensory perception of bitter taste	1	561.7×	0.012	RGS21
venous blood vessel morphogenesis	1	481.5×	0.012	CCM2
sensory perception of sweet taste	1	481.5×	0.012	RGS21
sensory perception of umami taste	1	481.5×	0.012	RGS21
pericardium development	1	374.5×	0.012	CCM2
endothelial tube morphogenesis	1	374.5×	0.012	CCM2
endothelium development	1	259.3×	0.014	CCM2
collagen-activated tyrosine kinase receptor signaling pathway	1	259.3×	0.014	COL4A2
regulation of androgen receptor signaling pathway	1	198.3×	0.017	KDM4C
blastocyst formation	1	153.2×	0.018	KDM4C
regulation of stem cell differentiation	1	153.2×	0.018	KDM4C
androgen receptor signaling pathway	1	140.4×	0.018	KDM4C
stress-activated MAPK cascade	1	140.4×	0.018	CCM2
transmission of nerve impulse	1	129.6×	0.018	RGS21
cell-cell junction organization	1	124.8×	0.018	CCM2
G protein-coupled receptor signaling pathway	2	14.5×	0.018	RGS21, RGS1
endodermal cell differentiation	1	99.1×	0.022	COL4A2
stem cell population maintenance	1	84.3×	0.023	KDM4C
regulation of angiogenesis	1	84.3×	0.023	CCM2
inner ear development	1	74.9×	0.025	CCM2
response to activity	1	64.8×	0.028	COL4A2
positive regulation of GTPase activity	1	55.2×	0.030	RGS1
cellular response to transforming growth factor beta stimulus	1	55.2×	0.030	COL4A2
vasculogenesis	1	51.1×	0.031	CCM2
DNA-templated transcription	1	44.9×	0.033	COL4A2
collagen fibril organization	1	44.9×	0.033	COL4A2

Therapeutics

Drugs indicated for this disease

2 approved, 7 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Mepolizumab	Approved (phase 4)
Rituximab	Approved (phase 4)
Avacopan	Phase 3 (in late-stage trials)
Azathioprine	Phase 3 (in late-stage trials)
Belimumab	Phase 3 (in late-stage trials)
Colchicine	Phase 3 (in late-stage trials)
Ibuprofen	Phase 3 (in late-stage trials)
Methotrexate	Phase 3 (in late-stage trials)
Prednisone	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Mycophenolate Mofetil.

Drug target analysis

Approved (phase 4): 0 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 4

Druggability breadth: 2 of 5 evidence-associated genes (40%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Top cohort targets by molecule count

Symbol	Molecules	Max phase
KDM4C	4	3
CCM2	0	0
COL4A2	0	0
RGS21	0	0
RGS1	0	0

Drugs targeting cohort genes (top 30)

Molecule	Max phase	Targets in cohort
TANESPIMYCIN	3	KDM4C
GELDANAMYCIN	2	KDM4C
ALVESPIMYCIN	2	KDM4C
ZAVONDEMSTAT	2	KDM4C

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 1.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

Symbol	Assays	Type breakdown
KDM4C	122	Binding:120, Functional:2

Cohort enzymes (BRENDA EC)

Symbol	EC numbers	Names
KDM4C	1.14.11.27, 1.14.11.66, 1.14.11.69	[histone H3]-dimethyl-L-lysine36 demethylase, [histone H3]-trimethyl-L-lysine9 demethylase, [histone H3]-trimethyl-L-lysine36 demethylase

Cohort genes with high screening signal

≥100 ChEMBL assays — a studied-ness signal; see Therapeutics for approved-drug status.

Symbol	ChEMBL assays
KDM4C	122

Pharmacogenomics

Cohort genes with a PharmGKB record: 5; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

4 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

Compound	Max phase	Cohort target (bioactivity)
TANESPIMYCIN	3	KDM4C
GELDANAMYCIN	2	KDM4C
ALVESPIMYCIN	2	KDM4C
ZAVONDEMSTAT	2	KDM4C

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	0
B	Phased (≥1) drug, not yet approved	1	KDM4C
C	Druggable family + PDB, no drug	0
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	4	CCM2, COL4A2, RGS21, RGS1

Undrugged target profiles

4 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

Symbol	ChEMBL assays	Drugged partners (top 3)
CCM2	0	—
COL4A2	0	—
RGS21	0	—
RGS1	0	—

Clinical trials & evidence

Clinical trials

Clinical trials: 98.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	63
PHASE2	12
PHASE3	7
PHASE4	6
PHASE1	4
PHASE2/PHASE3	3
PHASE1/PHASE2	2
EARLY_PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06763783	PHASE4	ENROLLING_BY_INVITATION	Vaccination Against Herpes Zoster in Patients With Inflammatory Rheumatic Diseases
NCT00128895	PHASE4	TERMINATED	Prevention of Relapses in Proteinase 3 (PR3)-Anti-neutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis
NCT00307671	PHASE4	COMPLETED	Treatment of Necrotizing Vasculitides for Patients Older Than 65 Years
NCT01151644	PHASE4	UNKNOWN	Safety and Efficacy of Anti-Pandemic H1N1 Vaccination in Rheumatic Diseases
NCT01405807	PHASE4	UNKNOWN	Alemtuzumab for ANCA Associated Refractory Vasculitis
NCT03762824	PHASE4	COMPLETED	Combined Pneumococcal Conjugate and Polysaccharide Vaccination in Inflammatory Rheumatic Disease
NCT01933724	PHASE3	ACTIVE_NOT_RECRUITING	The Assessment of Prednisone In Remission Trial (TAPIR) - Patient Centric Approach
NCT06321601	PHASE3	RECRUITING	Study to Evaluate Avacopan in Combination With a Rituximab or Cyclophosphamide-containing Regimen, in Children From 6 Years to < 18 Years of Age With AAV.
NCT00103792	PHASE3	COMPLETED	Mycophenolate Mofetil for Treatment of Relapses of Wegener’s Disease or Microscopic Polyangiitis (MPA)
NCT00104299	PHASE2/PHASE3	COMPLETED	Rituximab for the Treatment of Wegener’s Granulomatosis and Microscopic Polyangiitis
NCT00414128	PHASE2/PHASE3	COMPLETED	Clinical Trial of Mycophenolate Versus Cyclophosphamide in ANCA Vasculitis
NCT01257802	PHASE3	TERMINATED	GnRH-a for Ovarian Protection During CYC Therapy for Rheumatic Diseases
NCT01663623	PHASE3	COMPLETED	Belimumab in Remission of VASculitis
NCT03371095	PHASE3	COMPLETED	Induction Therapy With Anti-TNFα vs Cyclophosphamide in Severe Behçet Disease
NCT03692416	PHASE3	UNKNOWN	The Effect of Some Drugs Used in Treatment of Vasculitis on the Complement System in Children
NCT05236491	PHASE2/PHASE3	UNKNOWN	COvid-19 Vaccine Booster in Immunocompromised Rheumatic Diseases
NCT02638701	PHASE1/PHASE2	RECRUITING	Infliximab Therapy for Dolichoectactic Vertebrobasilar Aneurysms
NCT04629144	PHASE2	RECRUITING	Efficacy and Safety of Belimumab in the Treatment of Non-infectious Active Cryoglobulinemia Vasculitis Compared to Placebo. TRIBECA STUDY (Treatment nd BElimumab in Cryoglobulinemia Associated Vasculitis)
NCT00001155	PHASE2	COMPLETED	Treatment of Wegener’s Granulomatosis With Cyclophosphamide
NCT00001256	PHASE2	COMPLETED	Steroids and Methotrexate to Treat Systemic Vasculitis
NCT00001901	PHASE2	COMPLETED	Etanercept to Treat Wegener’s Granulomatosis
NCT00004357	PHASE2	COMPLETED	Absorption of Corticosteroids in Children With Juvenile Dermatomyositis
NCT00004567	PHASE2	COMPLETED	Comparison of Treatments to Maintain Disease Remission in Patients With Wegener’s Granulomatosis and Related Vasculitis Syndromes
NCT00005928	PHASE2	COMPLETED	Effects of Angiotensin-Converting Enzyme Inhibitor (Ramipril) Therapy on Blood Vessel Inflammation
NCT00029107	PHASE2	COMPLETED	Rituximab to Treat Hepatitis C-Associated Cryoglobulinemic Vasculitis
NCT01170936	PHASE2	COMPLETED	Ilaris® in Urticarial Vasculitis - Investigation of Treatment Responses
NCT01363388	PHASE2	COMPLETED	A Study to Evaluate the Safety and Efficacy of CCX168 in Subjects With ANCA-Associated Vasculitis
NCT02418273	PHASE1/PHASE2	WITHDRAWN	Denosumab for Glucocorticoid-treated Children With Rheumatic Disorders
NCT02556866	PHASE2	TERMINATED	Rituximab Plus Corticosteroids in Non-infectious Active Mixed Cryoglobulinemia Vasculitis
NCT03514979	PHASE2	UNKNOWN	Acquired Immunodeficiency in ANCA Associated Vasculitis
NCT00001761	PHASE1	COMPLETED	Phase I Trial of Recombinant Human Interleukin-10 (SCH 52000) in Patients With Wegener’s Granulomatosis
NCT00001764	PHASE1	COMPLETED	Mycophenolate Mofetil to Treat Wegener’s Granulomatosis and Related Vascular Inflammatory Conditions
NCT00278512	PHASE1	TERMINATED	Hematopoietic Stem Cell Support in Vasculitis
NCT05984251	PHASE1	COMPLETED	A Study to Evaluate the Safety, Tolerability, and Pharmacokinetics of CCX168 in Healthy Participants
NCT05263817	EARLY_PHASE1	UNKNOWN	A Clinical Study of CD19/BCMA CAR-T Cells in the Treatment of Refractory POEMS Syndrome, Amyloidosis, Autoimmune Hemolytic Anemia, and Vasculitis
NCT02280733	Not specified	RECRUITING	A Real-World Registry of Chronic Wounds and Ulcers
NCT02593565	Not specified	RECRUITING	Vasculitis Pregnancy Registry
NCT03004326	Not specified	RECRUITING	Clinical Transcriptomics in Systemic Vasculitis (CUTIS)
NCT03755245	Not specified	ENROLLING_BY_INVITATION	Biodistribution, Dosimetry and Performance of [68Ga]Ga-DOTA-Siglec-9 in Healthy and Patients With Rheumatoid Arthritis, Vasculitis or Pulmonary Sarcoidosis
NCT04006535	Not specified	RECRUITING	Whole Exome Sequencing of Familial and Pediatric Forms of Vasculitis

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
AZATHIOPRINE	4	4
AVACOPAN	4	3
CYCLOPHOSPHAMIDE ANHYDROUS	4	3
BELIMUMAB	4	2
ALEMTUZUMAB	4	1
CANAKINUMAB	4	1
METHOTREXATE	4	1
METHYLPREDNISOLONE	4	1
MYCOPHENOLATE MOFETIL	4	1
PREDNISONE	4	1
RAMIPRIL	4	1
STREPTOCOCCUS PNEUMONIAE POLYSACCHARIDE CONJUGATED TO CORYNEBACTERIUM DIPHTHERIAE CRM197	4	1
VARICELLA ZOSTER VIRUS ENVELOPE GLYCOPROTEIN E	4	1
BQ-123	2	1
INTERLEUKIN-10	2	1
CHEMBL15720	0	1
CHEMBL426	0	1