ORPHA:761
Immunoglobulin A vasculitis
Synonyms: Anaphylactoid purpura · Henoch-Schönlein purpura · IgA vasculitis · Purpura rheumatica · Rheumatoid purpura
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare, small-vessel vasculitis characterized by skin purpura, arthritis, abdominal and/or renal involvement, IgA tissue deposits (arterioles, capillaries, and venules) and circulating IgA immune complexes.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Immunoglobulin A vasculitis | Anaphylactoid purpura · Henoch-Schönlein purpura · IgA vasculitis · Purpura rheumatica · Rheumatoid purpura |
| fr | Vascularite à immunoglobulines A | Purpura anaphylactique · Purpura de Henoch-Schönlein · Purpura rhumatoïde · Vascularite à IgA |
| de | Immunoglobulin A-Vaskulitis | IgA-Vaskulitis · Purpura Schönlein-Henoch · Purpura rheumatica · Purpura, anaphylaktoide · Rheumatische Purpura · Schoenlein-Henoch-Purpura |
| es | Vasculitis por inmunoglobulina A | Púrpura anafilactoide · Púrpura de Henoch-Schönlein · Púrpura reumatoide · Púrpura reumática · Vasculitis IgA |
| it | Vasculite da immuglobuline A | Porpora anafilattoide · Porpora di Henoch-Schönlein · Porpora reumatica · Porpora reumatoide · Vasculite da IgA |
| nl | Immunoglobuline A-vasculitis | Anafylactoïde purpura · IgA-vasculitis · Purpura rheumatica · Purpura van Henoch-Schönlein · Reumatoïde purpura |
| pt | Vasculite Imunoglobulina A | Púrpura anafilática · Púrpura de Henoch-Schönlein · Púrpura reumatóide · Púrpura reumática · Vasculite IgA |
| pl | plamica Henocha i Schönleina | Czerwienica reumatyczna · plamica rzekomoanafilaktyczna · Zapalenie naczyń związane z immunoglobuliną A |
| cs | Imunoglobulin A vaskulitida | Anafylaktoidní purpura · Henochova-Schönleinova purpura · IgA vaskulitida · Revmatická purpura · Revmatoidní purpura · Henoch-Schönleinova purpura |
| uk | Васкуліт, асоційований з імуноглобуліном А | Анафілактоїдна пурпура · Пурпура Геноха-Шенлейна · Васкуліт IgA · Ревматична пурпура · Ревматоїдна пурпура |
| zh | IgA血管炎 | Henoch-Schönlein紫癜 |
| tr | İmmünoglobulin A vasküliti | IgA vasküliti · Purpura romatika · Romatoid purpura · Anafilaktoid purpura · Henoch-Schönlein purpurası |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D69.0 | NTBT | inclusion | validated |
| ICD-11 | 4A44.92 | E | specific | validated |
| MONDO | 0019167 | E | validated | |
| MeSH | D011695 | E | validated | |
| UMLS | C0034152 | E | validated | |
| GARD | 8204 | E | validated | |
| MedDRA | 10082960 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (4):
- ORPHA:156149Immune complex mediated vasculitis
rare systemic and rheumatological diseases; rare circulatory system diseases - ORPHA:280369Rare pediatric vasculitis
rare systemic or rheumatologic diseases of childhood - ORPHA:264709Secondary interstitial lung disease specific to childhood associated with a systemic vasculitis
rare respiratory diseases; rare transplant-related disorders - ORPHA:567560Systemic vasculitis associated with glomerulopathy
rare renal diseases; rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
8 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 6-9 / 10 000 | 55.9 / 100 000 | Korea, Republic of | Value and class | Validated | 36263029[PMID] |
| Annual incidence | 1-5 / 10 000 | 30 / 100 000 | France | Value and class | Validated | 36263029[PMID] |
| Annual incidence | 1-9 / 100 000 | 6.79 / 100 000 | Croatia | Value and class | Validated | 36263029[PMID] |
| Annual incidence | 1-5 / 10 000 | 13 / 100 000 | United Kingdom | Value and class | Validated | 36263029[PMID] |
| Annual incidence | 1-9 / 100 000 | 6.1 / 100 000 | Netherlands | Value and class | Validated | 36263029[PMID] |
| Annual incidence | 1-5 / 10 000 | 17.55 / 100 000 | Sweden | Value and class | Validated | 36263029[PMID] |
| Annual incidence | 1-9 / 100 000 | 12.9 / 100 000 | Taiwan, Province of China | Value and class | Validated | 36263029[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 36263029[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
34 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0002829 | Arthralgia | |
| HP:0000978 | Bruising susceptibility | |
| HP:0005244 | Gastrointestinal infarctions | |
| HP:0000790 | Hematuria | |
| HP:0002017 | Nausea and vomiting | |
| HP:0000979 | Purpura | |
| HP:0200039 | Pustule | |
| HP:0000988 | Skin rash | |
| HP:0002633 | Vasculitis | |
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0001369 | Arthritis | |
| HP:0010783 | Erythema | |
| HP:0001945 | Fever | |
| HP:0002383 | Infectious encephalitis | |
| HP:0002076 | Migraine | |
| HP:0003326 | Myalgia | |
| HP:0100796 | Orchitis | |
| HP:0200042 | Skin ulcer | |
| HP:0011276 | Vascular skin abnormality | |
| Occasional (29-5%) | ||
| HP:0100665 | Angioedema | |
| HP:0000969 | Edema | |
| HP:0100534 | Episcleritis | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0100820 | Glomerulopathy | |
| HP:0004374 | Hemiplegia/hemiparesis | |
| HP:0012733 | Macule | |
| HP:0001324 | Muscle weakness | |
| HP:0000648 | Optic atrophy | |
| HP:0000093 | Proteinuria | |
| HP:0000083 | Renal insufficiency | |
| HP:0002091 | Restrictive ventilatory defect | |
| HP:0001250 | Seizure | |
| HP:0001025 | Urticaria | |
Run the differential on the 10 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/761
- This record as JSON: /data/d/761.json