immunoglobulin A vasculitis
diseaseOn this page
Also known as allergic purpuraanaphylactoid purpuraHenoch Schonlein purpuraHSPIgA vasculitispurpura rheumaticapurpura, Schonlein-Henochrheumatoid purpuraSchoenlein-Henoch purpuravascular purpura
Summary
immunoglobulin A vasculitis (MONDO:0019167) is a disease and 16 clinical trials. Top therapeutic interventions include azathioprine, colchicine, and dapsone. A subtype of hypersensitivity vasculitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide) [Orphanet-validated]
- Phenotypes (HPO): 34
- Clinical trials: 16
Clinical features
Epidemiology
Prevalence records
7 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 6-9 / 10 000 | 55.9 | Korea, Republic of | Validated |
| Annual incidence | 1-5 / 10 000 | 30 | France | Validated |
| Annual incidence | 1-9 / 100 000 | 6.79 | Croatia | Validated |
| Annual incidence | 1-5 / 10 000 | 13 | United Kingdom | Validated |
| Annual incidence | 1-9 / 100 000 | 6.1 | Netherlands | Validated |
| Annual incidence | 1-5 / 10 000 | 17.55 | Sweden | Validated |
| Annual incidence | 1-9 / 100 000 | 12.9 | Taiwan, Province of China | Validated |
Signs & symptoms
Clinical features (HPO)
34 HPO clinical features (Orphanet curated; top 34 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0000790 | Hematuria | Very frequent (80-99%) |
| HP:0000978 | Bruising susceptibility | Very frequent (80-99%) |
| HP:0000979 | Purpura | Very frequent (80-99%) |
| HP:0000988 | Skin rash | Very frequent (80-99%) |
| HP:0002017 | Nausea and vomiting | Very frequent (80-99%) |
| HP:0002027 | Abdominal pain | Very frequent (80-99%) |
| HP:0002633 | Vasculitis | Very frequent (80-99%) |
| HP:0002829 | Arthralgia | Very frequent (80-99%) |
| HP:0005244 | Gastrointestinal infarctions | Very frequent (80-99%) |
| HP:0200039 | Pustule | Very frequent (80-99%) |
| HP:0001369 | Arthritis | Frequent (30-79%) |
| HP:0001945 | Fever | Frequent (30-79%) |
| HP:0002039 | Anorexia | Frequent (30-79%) |
| HP:0002076 | Migraine | Frequent (30-79%) |
| HP:0002383 | Infectious encephalitis | Frequent (30-79%) |
| HP:0003326 | Myalgia | Frequent (30-79%) |
| HP:0010783 | Erythema | Frequent (30-79%) |
| HP:0011276 | Vascular skin abnormality | Frequent (30-79%) |
| HP:0100796 | Orchitis | Frequent (30-79%) |
| HP:0200042 | Skin ulcer | Frequent (30-79%) |
| HP:0000083 | Renal insufficiency | Occasional (5-29%) |
| HP:0000093 | Proteinuria | Occasional (5-29%) |
| HP:0000648 | Optic atrophy | Occasional (5-29%) |
| HP:0000969 | Edema | Occasional (5-29%) |
| HP:0001025 | Urticaria | Occasional (5-29%) |
| HP:0001250 | Seizure | Occasional (5-29%) |
| HP:0001324 | Muscle weakness | Occasional (5-29%) |
| HP:0002091 | Restrictive ventilatory defect | Occasional (5-29%) |
| HP:0002239 | Gastrointestinal hemorrhage | Occasional (5-29%) |
| HP:0004374 | Hemiplegia/hemiparesis | Occasional (5-29%) |
| HP:0012733 | Macule | Occasional (5-29%) |
| HP:0100534 | Episcleritis | Occasional (5-29%) |
| HP:0100665 | Angioedema | Occasional (5-29%) |
| HP:0100820 | Glomerulopathy | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | immunoglobulin A vasculitis |
| Mondo ID | MONDO:0019167 |
| MeSH | D011695 |
| Orphanet | 761 |
| DOID | DOID:11123 |
| ICD-10-CM | D69.0 |
| ICD-11 | 1629105375 |
| NCIT | C34963 |
| SNOMED CT | 191306005, 86074002 |
| UMLS | C0034152 |
| MedGen | 48265 |
| GARD | 0008204 |
| Is cancer (heuristic) | no |
Also known as: allergic purpura · anaphylactoid purpura · Henoch Schonlein purpura · HSP · IgA vasculitis · purpura rheumatica · purpura, Schonlein-Henoch · rheumatoid purpura · Schoenlein-Henoch purpura · vascular purpura
Disease family
This is a subtype of hypersensitivity 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 › immune system disorder › hypersensitivity reaction disease › hypersensitivity vasculitis › immunoglobulin A vasculitis
Related subtypes (1): allergic cutaneous vasculitis
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
0 approved, 1 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Rituximab | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Dexamethasone, Leflunomide, Methylprednisolone, Mycophenolate Mofetil, Prednisone.
Clinical trials & evidence
Clinical trials
Clinical trials: 16.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 8 |
| PHASE3 | 3 |
| PHASE2 | 3 |
| PHASE4 | 1 |
| PHASE1/PHASE2 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT06676384 | PHASE4 | RECRUITING | Which of the Commonly Available and Approved Drugs in Addition to Standard of Care Can Significantly Improve the Slope of Estimated Glomerular Filtration Rate at Two Years When Compared to Standard of Care Alone in South-Asian Kidney Biopsy-proven Adult (≥18 Years) Primary IgA Nephropathy? |
| NCT05329090 | PHASE3 | ACTIVE_NOT_RECRUITING | Evaluation of Glucocorticoids Plus Rituximab in Patients with Newly-Diagnosed or Relapsing IgA Vasculitis |
| NCT07024563 | PHASE3 | RECRUITING | Study of Ravulizumab in Pediatric Participants With Primary IgAN |
| NCT04008316 | PHASE3 | UNKNOWN | Efficacy of Colchicine to Prevent Skin Relapses in Adult’s IgA Vasculitis |
| NCT02939573 | PHASE2 | RECRUITING | A Randomized Multicenter Study for Isolated Skin Vasculitis |
| NCT05003986 | PHASE2 | RECRUITING | Study of Sparsentan Treatment in Pediatrics With Proteinuric Glomerular Diseases |
| NCT06948019 | PHASE1/PHASE2 | NOT_YET_RECRUITING | Safety and Efficacy of AAV9/AP4B1 (BFB-101) For Patients With AP4B1-related Hereditary Spastic Paraplegia Type 47 (SPG47) |
| NCT05168475 | PHASE2 | TERMINATED | Biologics in Refractory Vasculitis: A Trial of Biologic Therapy for Refractory Primary Non-ANCA Associated Vasculitis |
| NCT02593565 | Not specified | RECRUITING | Vasculitis Pregnancy Registry |
| NCT02967068 | Not specified | RECRUITING | VCRC Tissue Repository |
| NCT03004326 | Not specified | RECRUITING | Clinical Transcriptomics in Systemic Vasculitis (CUTIS) |
| NCT05937880 | Not specified | RECRUITING | Leflunomide for Henoch-Schonlein Purpura |
| NCT07354503 | Not specified | NOT_YET_RECRUITING | Telitacicept in the Treatment of Pediatric IgA Vasculitis-Associated Nephritis |
| NCT03410290 | Not specified | COMPLETED | Journey of Patients With Vasculitis From First Symptom to Diagnosis |
| NCT04655378 | Not specified | COMPLETED | Validation of the IgA1 Detection Method With Gradient Glycosylation by Mass Spectrometry as a Potential Marker of Renal Involvement in Pediatric Rheumatoid Purpura |
| NCT05737056 | Not specified | COMPLETED | Rheumatoid Purpura in Children: a Multicenter Observational Study of Pediatric Emergency Department Management and Follow-up by Primary Care Physicians |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| AZATHIOPRINE | 4 | 1 |
| COLCHICINE | 4 | 1 |
| DAPSONE | 4 | 1 |
| HYDROXYCHLOROQUINE | 4 | 1 |
| LEFLUNOMIDE | 4 | 1 |
| PREDNISOLONE | 4 | 1 |
| RAVULIZUMAB | 4 | 1 |
| SPARSENTAN | 4 | 1 |