ORPHA:183
Eosinophilic granulomatosis with polyangiitis
Synonyms: Churg-Strauss syndrome · EGPA · Granulomatous allergic angiitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
A rare systemic vasculitis of small vessels characterized by asthma, blood and tissue eosinophilia and vasculitis manifestations.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Eosinophilic granulomatosis with polyangiitis | Churg-Strauss syndrome · EGPA · Granulomatous allergic angiitis |
| fr | Granulomatose éosinophilique avec polyangéite | Angéite allergique granulomateuse · EGPA · Syndrome de Churg-Strauss |
| de | Eosinophile Granulomatose mit Polyangiitis | Angiitis, allergische granulomatöse · Churg-Strauss-Syndrom · EGPA |
| es | Granulomatosis eosinofílica con poliangeítis | Síndrome de Churg-Strauss |
| it | Granulomatosi eosinofilica con poliangioite | Sindrome di Churg-Strauss |
| nl | Eosinofiele granulomatose met polyangiitis | EGPA · Granulomateuze allergische angiitis · Syndroom van Churg-Strauss |
| pt | Granulomatose eosinofílica com poliangeíte | Doença de Churg-Strauss |
| pl | Eozynofilowa ziarniniakowatość z zapaleniem naczyń | Alergiczne ziarniniakowe zapalenie naczyń · EGPA · Zespół Churga i Straussa |
| cs | Eozinofilní granulomatóza s polyangiitidou | EGPA · Alergická granulomatózní angiitida · Churgův-Straussové syndrom · Churg-Straussové syndrom |
| uk | Еозинофільний гранулематоз з поліангіїтом | Синдром Чарга-Стросса · EGPA · Гранулематозний алергічний ангіїт |
| zh | 嗜酸性肉芽肿性血管炎 | 肉芽肿性过敏性血管炎 |
| tr | Polianjiitli eozinofilik granülomatoz | EGPA · Churg-Strauss sendromu · Granülomatöz alerjik anjit |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M30.1 | E | specific | validated |
| ICD-11 | 4A44.A2 | E | specific | validated |
| MONDO | 0015943 | E | validated | |
| MeSH | D015267 | E | validated | |
| UMLS | C0008728 | E | validated | |
| GARD | 6111 | E | validated | |
| MedDRA | 10048594 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (4):
- ORPHA:156152Anti-neutrophil cytoplasmic antibody-associated vasculitis
rare respiratory diseases; rare systemic and rheumatological diseases; rare circulatory system diseases; rare systemic or rheumatologic diseases of childhood; rare transplant-related disorders - ORPHA:324767Non-familial rare disease with dilated cardiomyopathy
rare cardiac diseases; rare transplant-related disorders - ORPHA:209007Systemic inflammatory disease associated with an acquired peripheral neuropathy
rare neurological diseases - ORPHA:567560Systemic vasculitis associated with glomerulopathy
rare renal diseases; rare transplant-related disorders
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
19 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 1.56 / 100 000 | Europe | Value and class | Validated | ORPHANET_25805746[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.09 / 100 000 | Sweden | Value and class | Validated | 19797309[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.13 / 100 000 | France | Value and class | Validated | 20039171[PMID]_25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.4 / 100 000 | Sweden | Value and class | Validated | 17553910[PMID]_25805746[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.09 / 100 000 | United Kingdom | Value and class | Validated | 21798892[PMID]_25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.3 / 100 000 | Norway | Value and class | Validated | 9805179[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.09 / 100 000 | Spain | Value and class | Validated | 11156552[PMID] |
| Point prevalence | 1-9 / 100 000 | 2.23 / 100 000 | Australia | Value and class | Validated | 18771432[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.18 / 100 000 | Europe | Value and class | Validated | ORPHANET_19797309[PMID]_18565978[PMID]_11156552[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.5 / 100 000 | Worldwide | Value and class | Validated | 18771432[PMID]_9805179[PMID]_17553910[PMID]_14872461[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | Norway | Value and class | Validated | 11760724[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.11 / 100 000 | Germany | Value and class | Validated | 15696553[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.13 / 100 000 | Lithuania | Value and class | Validated | 15647446[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.12 / 100 000 | France | Value and class | Validated | 20039171[PMID]_25805746[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.06 / 100 000 | Turkey | Value and class | Validated | 25805746[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.014 / 100 000 | Peru | Value and class | Validated | 25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 2.4 / 100 000 | Germany | Value and class | Validated | 24425780[PMID]_25805746[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.78 / 100 000 | Japan | Value and class | Validated | 24289197[PMID]_25805746[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.4 / 100 000 | United States | Value and class | Validated | 28881446[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
6
HPO phenotypes
66 annotations · validated 2024-10-07| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002099 | Asthma | |
| HP:0002960 | Autoimmunity | |
| HP:0007009 | Central nervous system degeneration | |
| HP:0012649 | Increased inflammatory response | |
| HP:0001880 | Increased total eosinophil count | |
| HP:0009830 | Peripheral neuropathy | |
| HP:0002113 | Pulmonary infiltrates | |
| HP:0000979 | Purpura | |
| HP:0000246 | Sinusitis | |
| HP:0001025 | Urticaria | |
| HP:0002633 | Vasculitis | |
| HP:0001824 | Weight loss | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001697 | Abnormal pericardium morphology | |
| HP:0002103 | Abnormality of the pleura | |
| HP:0006536 | Airway obstruction | |
| HP:0033559 | Anti-myeloperoxidase antibody positivity | |
| HP:0034104 | Anti-neutrophil elastase antibody positivity | |
| HP:0033557 | Anti-proteinase 3 antibody positivity | |
| HP:0002829 | Arthralgia | |
| HP:0032230 | Cytoplasmic antineutrophil antibody positivity | |
| HP:0002015 | Dysphagia | |
| HP:0032071 | Eosinophilic pneumonia | |
| HP:0012378 | Fatigue | |
| HP:0001288 | Gait disturbance | |
| HP:0000790 | Hematuria | |
| HP:0000822 | Hypertension | |
| HP:0001639 | Hypertrophic cardiomyopathy | |
| HP:0001053 | Hypopigmented skin patches | |
| HP:0003212 | Increased circulating IgE concentration | |
| HP:0032018 | Multiple mononeuropathy | |
| HP:0002017 | Nausea and vomiting | |
| HP:0002923 | Rheumatoid factor positive | |
| HP:0000988 | Skin rash | |
| HP:0001970 | Tubulointerstitial nephritis | |
| HP:0004936 | Venous thrombosis | |
| Occasional (29-5%) | ||
| HP:0001063 | Acrocyanosis | |
| HP:0001369 | Arthritis | |
| HP:0001635 | Congestive heart failure | |
| HP:0012735 | Cough | |
| HP:0006824 | Cranial nerve paralysis | |
| HP:0008653 | Crescentic glomerulonephritis | |
| HP:6000070 | Cutaneous granuloma | |
| HP:0000965 | Cutis marmorata | |
| HP:0100584 | Endocarditis | |
| HP:0001945 | Fever | |
| HP:0002020 | Gastroesophageal reflux | |
| HP:0032064 | Gastrointestinal eosinophilia | |
| HP:0100820 | Glomerulopathy | |
| HP:0004374 | Hemiplegia/hemiparesis | |
| HP:0002105 | Hemoptysis | |
| HP:0005214 | Intestinal obstruction | |
| HP:0002024 | Malabsorption | |
| HP:0003326 | Myalgia | |
| HP:0001658 | Myocardial infarction | |
| HP:0012819 | Myocarditis | |
| HP:0100614 | Myositis | |
| HP:0100582 | Nasal polyposis | |
| HP:0200034 | Papule | |
| HP:0002202 | Pleural effusion | |
| HP:0000093 | Proteinuria | |
| HP:0006535 | Recurrent intrapulmonary hemorrhage | |
| HP:0000083 | Renal insufficiency | |
| HP:0002093 | Respiratory insufficiency | |
| HP:0001482 | Subcutaneous nodule | |
| HP:0002326 | Transient ischemic attack | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 30725784[PMID]
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/183
- This record as JSON: /data/d/183.json