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Atlas / Disease / Microscopic polyangiitis

Microscopic polyangiitis

disease
On this page

Also known as Micropolyangiitismicroscopic polyarteritisMPA

Summary

Microscopic polyangiitis (MONDO:0019124) is a disease with 1 GWAS associations across 1 studies and 52 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous, azathioprine, and rituximab. A subtype of anti-neutrophil cytoplasmic antibody-associated vasculitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: 1-9 / 100 000 (Worldwide) [Orphanet-validated]
  • GWAS associations: 1
  • Phenotypes (HPO): 55
  • Clinical trials: 52

Clinical features

Epidemiology

Prevalence records

21 prevalence record(s), Orphanet, top 20 (validated / broadest geography first):

TypeClassValueGeographyValidation
Point prevalence1-9 / 100 0004.2843WorldwideValidated
Annual incidence1-9 / 100 0001EuropeValidated
Annual incidence1-9 / 1 000 0000.26GermanyValidated
Annual incidence1-9 / 100 0001.16SpainValidated
Annual incidence1-9 / 1 000 0000.5AustraliaValidated
Annual incidence1-9 / 1 000 0000.27NorwayValidated
Annual incidence1-9 / 100 0002.4KuwaitValidated
Annual incidence1-9 / 100 0001.82JapanValidated
Annual incidence1-9 / 1 000 0000.71CanadaValidated
Annual incidence1-9 / 100 0001.02GreeceValidated
Annual incidence1-9 / 1 000 0000.3LithuaniaValidated
Annual incidence1-9 / 100 0001.01SwedenValidated
Annual incidence1-9 / 100 0001.6United StatesValidated
Point prevalence1-9 / 100 0009.4SwedenValidated
Point prevalence1-9 / 100 0002.5FranceValidated
Point prevalence1-9 / 100 0003.7New ZealandValidated
Point prevalence1-9 / 100 0006.3United KingdomValidated
Point prevalence1-9 / 100 0002.8GermanyValidated
Point prevalence1-9 / 100 0003.91AustraliaValidated
Point prevalence1-9 / 100 0001.38JapanValidated

Signs & symptoms

Clinical features (HPO)

55 HPO clinical features (Orphanet curated; top 50 by frequency):

HPO IDTermFrequency
HP:0000083Renal insufficiencyVery frequent (80-99%)
HP:0000099GlomerulonephritisVery frequent (80-99%)
HP:0000790HematuriaVery frequent (80-99%)
HP:0000988Skin rashVery frequent (80-99%)
HP:0001945FeverVery frequent (80-99%)
HP:0002105HemoptysisVery frequent (80-99%)
HP:0002633VasculitisVery frequent (80-99%)
HP:0002960AutoimmunityVery frequent (80-99%)
HP:0008653Crescentic glomerulonephritisVery frequent (80-99%)
HP:0010783ErythemaVery frequent (80-99%)
HP:0012649Increased inflammatory responseVery frequent (80-99%)
HP:0100520OliguriaVery frequent (80-99%)
HP:0100820GlomerulopathyVery frequent (80-99%)
HP:0001903AnemiaFrequent (30-79%)
HP:0001933Subcutaneous hemorrhageFrequent (30-79%)
HP:0002014DiarrheaFrequent (30-79%)
HP:0002017Nausea and vomitingFrequent (30-79%)
HP:0002027Abdominal painFrequent (30-79%)
HP:0002239Gastrointestinal hemorrhageFrequent (30-79%)
HP:0002586PeritonitisFrequent (30-79%)
HP:0002829ArthralgiaFrequent (30-79%)
HP:0003326MyalgiaFrequent (30-79%)
HP:0003565Elevated erythrocyte sedimentation rateFrequent (30-79%)
HP:0004396Poor appetiteFrequent (30-79%)
HP:0004936Venous thrombosisFrequent (30-79%)
HP:0005244Gastrointestinal infarctionsFrequent (30-79%)
HP:0012378FatigueFrequent (30-79%)
HP:0012614Abnormal urine cytologyFrequent (30-79%)
HP:0032018Multiple mononeuropathyFrequent (30-79%)
HP:0032230Cytoplasmic antineutrophil antibody positivityFrequent (30-79%)
HP:0033505Livedo reticularisFrequent (30-79%)
HP:0033557Anti-proteinase 3 antibody positivityFrequent (30-79%)
HP:0033559Anti-myeloperoxidase antibody positivityFrequent (30-79%)
HP:0034104Anti-neutrophil elastase antibody positivityFrequent (30-79%)
HP:0200042Skin ulcerFrequent (30-79%)
HP:0000246SinusitisOccasional (5-29%)
HP:0000421EpistaxisOccasional (5-29%)
HP:0000554UveitisOccasional (5-29%)
HP:0000965Cutis marmorataOccasional (5-29%)
HP:0001369ArthritisOccasional (5-29%)
HP:0001482Subcutaneous noduleOccasional (5-29%)
HP:0001635Congestive heart failureOccasional (5-29%)
HP:0001701PericarditisOccasional (5-29%)
HP:0001733PancreatitisOccasional (5-29%)
HP:0001824Weight lossOccasional (5-29%)
HP:0003401ParesthesiaOccasional (5-29%)
HP:0008046Abnormal retinal vascular morphologyOccasional (5-29%)
HP:0009830Peripheral neuropathyOccasional (5-29%)
HP:0011675ArrhythmiaOccasional (5-29%)
HP:0025188Retinal vasculitisOccasional (5-29%)

Identifiers

Disease identifiers

FieldValue
Canonical namemicroscopic polyangiitis
Mondo IDMONDO:0019124
EFOEFO:1000784
MeSHD055953
Orphanet727
ICD-10-CMM31.7
ICD-11999231798
NCITC70549
SNOMED CT239928004
UMLSC2347126
MedGen389393
GARD0003652
MedDRA10063344
Is cancer (heuristic)no

Also known as: Micropolyangiitis · microscopic polyarteritis · MPA

Data availability: 1 GWAS association (1 study).

Disease family

This is a subtype of anti-neutrophil cytoplasmic antibody-associated 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 › microscopic polyangiitis

Related subtypes (1): granulomatosis with polyangiitis

Genetics & variants

GWAS landscape

1 GWAS associations across 1 studies. Top hits map to 0 distinct genes (as reported by GWAS).

Top associations by p-value

rsIDp-valueGeneRisk alleleOdds ratio
rs803213793e-08PRPF19P1 - CDH19?

Top studies (by case count)

StudyLead authorYearCasesControlsTitle
GCST90651630Liu TY2025159228,546Diversity and longitudinal records: Genetic architecture of disease associations and polygenic risk in the Taiwanese Han population.

Variant details and genetic-evidence tiers

Tier distribution (top 50 variants)

TierVariants
Tier 1: coding0
Tier 2: splice/UTR0
Tier 3: regulatory0
Tier 4: intronic/intergenic1

MAF distribution

BucketVariants
common (>=0.05)0
low_freq (0.01-0.05)0
rare (<0.01)0
unknown1

Functional consequences

ConsequenceCount
intergenic_variant1

Top variants

rsIDChrPosAllelesMAFConsequenceGenep-valueTier
rs803213791866431416A>Gintergenic_variantPRPF19P1 - CDH193e-08Tier 4: intronic/intergenic

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

2 approved, 4 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

DrugDevelopment status
AvacopanApproved (phase 4)
RituximabApproved (phase 4)
AzathioprinePhase 3 (in late-stage trials)
MethylprednisolonePhase 3 (in late-stage trials)
Mycophenolate MofetilPhase 3 (in late-stage trials)
PrednisonePhase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Blisibimod, Infliximab, Prednisolone, Vilobelimab.

Clinical trials & evidence

Clinical trials

Clinical trials: 52.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified24
PHASE48
PHASE37
PHASE27
PHASE2/PHASE33
PHASE12
PHASE1/PHASE21

Top trials by phase / activity

NCTPhaseStatusTitle
NCT07087912PHASE4RECRUITINGSafety and Immunogenicity of the Live Attenuated Tetravalent Butantan-Dengue Vaccine in Autoimmune Rheumatic Diseases
NCT07451847PHASE4NOT_YET_RECRUITINGComparison 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.
NCT00307671PHASE4COMPLETEDTreatment of Necrotizing Vasculitides for Patients Older Than 65 Years
NCT00400075PHASE4UNKNOWNCHUSPAN PAN BP Treatment of Polyarteritis Nodosa and Microscopic Polyangiitis Without Poor-Prognosis Factors
NCT01405807PHASE4UNKNOWNAlemtuzumab for ANCA Associated Refractory Vasculitis
NCT02169219PHASE4COMPLETEDPilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis
NCT02198248PHASE4UNKNOWNLow-dose Glucocorticoid Vasculitis Induction Study
NCT04316494PHASE4TERMINATEDHydroxychloroquine in ANCA Vasculitis Evaluation
NCT06983821PHASE3RECRUITINGSafe Effective Therapy With Low-Dose Glucocorticoid in ANCA-Associated Vasculitis (SAFE-LOW)
NCT00104299PHASE2/PHASE3COMPLETEDRituximab for the Treatment of Wegener’s Granulomatosis and Microscopic Polyangiitis
NCT00430105PHASE2/PHASE3COMPLETEDPulse Versus Continuous Cyclophosphamide for Induction of Remission in ANCA-Associated Vasculitides
NCT00647166PHASE3COMPLETEDAssociation Corticosteroid/Azathioprine in Microscopic Polyangiitis/ Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome)
NCT00748644PHASE3COMPLETEDEfficacy Study of Two Treatments in the Remission of Vasculitis
NCT00987389PHASE3COMPLETEDPlasma Exchange and Glucocorticoids for Treatment of Anti-Neutrophil Cytoplasm Antibody (ANCA) - Associated Vasculitis
NCT01408836PHASE2/PHASE3TERMINATEDPlasma Exchange for Renal Vasculitis
NCT01697267PHASE3COMPLETEDRituximab Vasculitis Maintenance Study
NCT01731561PHASE3COMPLETEDComparison Study of Two Rituximab Regimens in the Remission of ANCA Associated Vasculitis
NCT03920722PHASE3COMPLETEDEfficacy and Safety of Rituximab in the Treatment of Good Prognosis Microscopic Polyangiitis
NCT06350110PHASE1/PHASE2RECRUITINGFourth-gen CAR T Cells Targeting BCMA/CD19 for Refractory Systemic Lupus Erythematosus (SLE)
NCT00751517PHASE2UNKNOWNCyclophosphamide Versus Methotrexate for Remission Maintenance in Systemic Necrotizing Vasculitides
NCT00753103PHASE2COMPLETEDAnti-Cytokine Therapy for Vasculitis
NCT01598857PHASE2WITHDRAWNBIANCA-SC: A Study of the Efficacy, Safety, and Tolerability of Blisibimod in Addition to Methotrexate During Induction of Remission in Subjects With ANCA-Associated Small Vessel Vasculitis
NCT03482479PHASE2COMPLETEDLow Dose Naltrexone to Improve Physical Health in Patients With Vasculitis
NCT03712345PHASE2TERMINATEDSafety and Efficacy Study of IFX-1 in add-on to Standard of Care in GPA and MPA
NCT03895801PHASE2COMPLETEDStudy of IFX-1 to Replace Steroids in Patients With Granulomatosis With Polyangiitis and Microscopic Polyangiitis.
NCT05376319PHASE2TERMINATEDPR3-AAV Resilient Remission or PRRR
NCT06294236PHASE1ACTIVE_NOT_RECRUITINGStudy Evaluating SC291 in Subjects With Severe r/r B-cell Mediated Autoimmune Diseases (GLEAM)
NCT00405860PHASE1COMPLETEDCellCept in p-ANCA Vasculitis
NCT01241305Not specifiedRECRUITINGOne-Time DNA Study for Vasculitis
NCT02593565Not specifiedRECRUITINGVasculitis Pregnancy Registry
NCT02967068Not specifiedRECRUITINGVCRC Tissue Repository
NCT03004326Not specifiedRECRUITINGClinical Transcriptomics in Systemic Vasculitis (CUTIS)
NCT06758271Not specifiedRECRUITINGSpecial Drug Use-results Survey for Long-term Use（Avacopan）
NCT00307593Not specifiedCOMPLETEDRATTRAP: Infliximab Versus Rituximab in Systemic Necrotizing Vasculitides
NCT00315393Not specifiedCOMPLETEDLongitudinal Protocol for Granulomatosis With Polyangiitis (Wegener’s) and Microscopic Polyangiitis
NCT01066208Not specifiedUNKNOWNAmerican College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis
NCT01586858Not specifiedTERMINATEDRituximab for ANCA-associated Vasculitis (RAVE) Long-Term Follow-Up Study
NCT01613599Not specifiedCOMPLETEDAn Observational Study of The Safety of MabThera/Rituxan (Rituximab) in Participants With Granulomatosis With Polyangiitis (Wegener’s) or Microscopic Polyangiitis
NCT01729624Not specifiedUNKNOWNPRO Development for ANCA Associated Vasculitis
NCT02006134Not specifiedUNKNOWNPediatric Vasculitis Initiative

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
CYCLOPHOSPHAMIDE ANHYDROUS410
AZATHIOPRINE47
RITUXIMAB43
ALEMTUZUMAB41
AVACOPAN41
HYDROXYCHLOROQUINE41
METHOTREXATE41
METHYLPREDNISOLONE41
MYCOPHENOLATE MOFETIL41
PREDNISONE41
BLISIBIMOD31
VILOBELIMAB31
CHEMBL1572001
CHEMBL42601

About this page

Generated deterministically by Sugi Atlas from primary biomedical databases via BioBTree. Every record on this page traces to a primary source.

Generated
Atlas version
v1.1.2
BioBTree
v2.0.2

Sources 24

This page pulls from 24 datasets indexed by BioBTree:

chembl_moleculecivic_evidenceclinical_trialsclinvardbsnpefogardgenccgwasgwas_studyhgncicd10cmicd11meddramedgenmeshmimmondomondochildmondoparentncitorphanetsctidumls