Buerger disease
Synonyms: Thromboangiitis obliterans
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare systemic disease characterized by inflammatory, non-necrotizing, non-atherosclerotic, occlusive vascular disease with thrombosis and recanalization affecting small and medium sized arteries and veins of upper and lower extremities.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Buerger disease | Thromboangiitis obliterans |
| fr | Maladie de Buerger | Thromboangéite oblitérante |
| de | Buerger-Krankheit | Thrombangiitis obliterans |
| es | Enfermedad de Buerger | Tromboangitis obliterante |
| it | Malattia di Buerger | Tromboangioite obliterante |
| nl | Ziekte van Buerger | = en |
| pt | Doença Buerger | |
| pl | Choroba Buergera | Zakrzepowo-zatorowe zapalnie naczyń |
| cs | Buergerova choroba | Tromboangiitis obliterans |
| uk | Хвороба Бюргера | Облітеруючий тромбоангіїт |
| zh | Buerger病 | 血栓闭塞性脉管炎 |
| tr | Buerger hastalığı | Tromboangiitis obliterans |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 211480 | E | validated | |
| ICD-10 | I73.1 | E | specific | validated |
| ICD-11 | 4A44.8 | E | specific | validated |
| MONDO | 0008889 | E | validated | |
| MeSH | D013919 | E | validated | |
| UMLS | C0040021 | E | validated | |
| GARD | 5969 | E | validated | |
| MedDRA | 10043540 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:156143Predominantly medium-vessel vasculitis
rare systemic and rheumatological diseases; rare circulatory system diseases - ORPHA:567560Systemic vasculitis associated with glomerulopathy
rare renal diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 16 / 100 000 | Worldwide | Value and class | Validated | [EXPERT] |
| Point prevalence | 1-5 / 10 000 | 10 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2015[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
17 annotations · validated 2023-09-21| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0004420 | Arterial thrombosis | |
| HP:0100758 | Gangrene | |
| HP:0000763 | Sensory neuropathy | |
| HP:0200042 | Skin ulcer | |
| HP:0002633 | Vasculitis | |
| Frequent (79-30%) | ||
| HP:0001063 | Acrocyanosis | |
| HP:0002829 | Arthralgia | |
| HP:0033402 | Digital ischemia | |
| HP:0004417 | Intermittent claudication | |
| HP:0033505 | Livedo reticularis | |
| HP:0003401 | Paresthesia | |
| HP:0030880 | Raynaud phenomenon | |
| Occasional (29-5%) | ||
| HP:0031006 | Acroparesthesia | |
| HP:0000975 | Hyperhidrosis | |
| HP:0100785 | Insomnia | |
| HP:0002638 | Superficial thrombophlebitis | |
| Very rare (<4-1%) | ||
| HP:0025324 | Arterial occlusion | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 28613608[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/36258
- This record as JSON: /data/d/36258.json