ORPHA:767
Polyarteritis nodosa
Synonyms: Küssmaul-Maier disease · PAN · Periarteritis nodosa
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare, clinically heterogeneous, systemic disease characterized by necrotizing inflammatory lesions affecting medium-sized blood vessels. It most commonly affects skin, joints, peripheral nerves and the gastrointestinal tract.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Polyarteritis nodosa | Küssmaul-Maier disease · PAN · Periarteritis nodosa |
| fr | Périartérite noueuse | Maladie de Küssmaul-Maier · PAN · Panartérite noueuse · Polyartérite noueuse |
| de | Polyarteriitis nodosa | Küssmaul-Maier-Krankheit · PAN |
| es | Poliarteritis nodosa | Enfermedad de Küssmaul-Maier · PAN · Periarteritis nodosa |
| it | Poliarterite nodosa | Malattia di Küssmaul-Maier · PAN · Periartrite nodosa |
| nl | Polyarteriitis nodosa | PAN · Periarteriitis nodosa · Ziekte van Küssmaul-Maier |
| pt | Poliarterite nodosa | |
| pl | Guzkowe zapalenie tętnic | Choroba Küssmaula i Maiera · PAN |
| cs | Polyarteritis nodosa | Küssmaulova-Maierova nemoc · PAN · Periarteritis nodosa · Küssmaul-Maierova nemoc |
| uk | Вузликовий поліартеріїт | Хвороба Кюссмауля-Майєра · PAN · Вузликовий периартеріїт |
| zh | 结节性多动脉炎 | Küssmaul-Maier 综合征 |
| tr | Poliarteritis nodosa | TAVA · Periarteritis nodoza · Küssmaul-Maier hastalığı |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M30.0 | E | specific | validated |
| ICD-11 | 4A44.4 | E | specific | validated |
| MONDO | 0019170 | E | validated | |
| MeSH | D010488 | E | validated | |
| UMLS | C0031036 | E | validated | |
| GARD | 7360 | E | validated | |
| MedDRA | 10036024 | 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:156143Predominantly medium-vessel vasculitis
rare systemic and rheumatological diseases; rare circulatory system diseases - ORPHA:280369Rare pediatric vasculitis
rare systemic or rheumatologic diseases of childhood - 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
Codes that Orphanet moved or referred to this one:
- ORPHA:93564OBSOLETE: Pediatric polyarteritis nodosa referred to · inactive
4
Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
9 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 3.16 / 100 000 | Europe | Value and class | Validated | 9805179[PMID]_14872461[PMID]_17553910[PMID]_ORPHANET |
| Point prevalence | 1-9 / 100 000 | 3.1 / 100 000 | Sweden | Value and class | Validated | 17553910[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.9 / 100 000 | United Kingdom | Value and class | Not yet validated | 10693883[PMID]_11156552[PMID] |
| Point prevalence | 1-9 / 100 000 | 3.07 / 100 000 | France | Value and class | Validated | 14872461[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.11 / 100 000 | Australia | Value and class | Validated | 18771432[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.62 / 100 000 | Spain | Value and class | Validated | 11156552[PMID] |
| Point prevalence | 1-9 / 100 000 | 3.3 / 100 000 | Norway | Value and class | Validated | 9805179[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | Norway | Value and class | Not yet validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.04 / 100 000 | Germany | Value and class | Validated | 15696553[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.
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HPO phenotypes
28 annotations · validated 2019-05-15| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0011121 | Abnormal skin morphology | |
| Frequent (79-30%) | ||
| HP:0000077 | Abnormality of the kidney | |
| HP:0002829 | Arthralgia | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0001945 | Fever | |
| HP:0033260 | Livedo racemosa | |
| HP:0003326 | Myalgia | |
| HP:0009830 | Peripheral neuropathy | |
| HP:0005764 | Polyarticular arthritis | |
| HP:0031003 | Polyneuritis | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0030680 | Abnormal cardiovascular system morphology | |
| HP:0002088 | Abnormal lung morphology | |
| HP:0011024 | Abnormality of the gastrointestinal tract | |
| HP:0000707 | Abnormality of the nervous system | |
| HP:0000965 | Cutis marmorata | |
| HP:0010783 | Erythema | |
| HP:0000822 | Hypertension | |
| HP:0002011 | Morphological central nervous system abnormality | |
| HP:0001701 | Pericarditis | |
| HP:0030880 | Raynaud phenomenon | |
| HP:0003390 | Sensory axonal neuropathy | |
| HP:0200042 | Skin ulcer | |
| HP:0001482 | Subcutaneous nodule | |
| Very rare (<4-1%) | ||
| HP:0000478 | Abnormality of the eye | |
| HP:0001638 | Cardiomyopathy | |
| HP:0002102 | Pleuritis | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 29955757[PMID]_30114520[PMID]_30418125[PMID]_30952413[PMID]_26876385[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/767
- This record as JSON: /data/d/767.json