Polyarteritis nodosa
General Information (adopted from Orphanet):
| Synonyms, Signs: |
Periarteritis nodosa Küssmaul-Maier disease |
| Number of Symptoms | 58 |
| OrphanetNr: | 767 |
| OMIM Id: |
|
| ICD-10: |
M30.0 |
| UMLs: |
C0031036 |
| MeSH: |
D010488 |
| MedDRA: |
10036024 |
| Snomed: |
155441006 |
Prevalence, inheritance and age of onset:
| Prevalence: | 3.1 of 100 000 [Orphanet] |
| Inheritance: |
Autosomal recessive Not applicable [Orphanet] |
| Age of onset: |
All ages [Orphanet] |
Disease classification (adopted from Orphanet):
| Parent Diseases: |
Predominantly medium-vessel vasculitis
-Rare circulatory system disease -Rare systemic or rheumatologic disease Secondary glomerular disease -Rare renal disease Systemic inflammatory disease associated with an acquired peripheral neuropathy -Rare neurologic disease |
Symptom Information:
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(HPO:0000112) | Nephropathy | Very frequent [Orphanet] | 92 / 7739 | |||
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(HPO:0000071) | Ureteral stenosis | Occasional [Orphanet] | 9 / 7739 | |||
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(HPO:0000083) | Renal insufficiency | Very frequent [Orphanet] | 232 / 7739 | |||
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(HPO:0100796) | Orchitis | Very frequent [Orphanet] | 7 / 7739 | |||
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(HPO:0008046) | Abnormality of the retinal vasculature | Occasional [Orphanet] | 41 / 7739 | |||
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(HPO:0100533) | Inflammatory abnormality of the eye | Occasional [Orphanet] | 70 / 7739 | |||
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(HPO:0000541) | Retinal detachment | Occasional [Orphanet] | 87 / 7739 | |||
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(HPO:0002071) | Abnormality of extrapyramidal motor function | Occasional [Orphanet] | 76 / 7739 | |||
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(HPO:0007256) | Abnormal pyramidal signs | Very frequent [Orphanet] | 116 / 7739 | |||
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(HPO:0000763) | Sensory neuropathy | Very frequent [Orphanet] | 78 / 7739 | |||
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(HPO:0000708) | Behavioral abnormality | Frequent [Orphanet] | 212 / 7739 | |||
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(HPO:0002315) | Headache | Very frequent [Orphanet] | 175 / 7739 | |||
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(HPO:0001250) | Seizures | Frequent [Orphanet] | 1245 / 7739 | |||
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(HPO:0009830) | Peripheral neuropathy | Very frequent [Orphanet] | 206 / 7739 | |||
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(HPO:0004374) | Hemiplegia/hemiparesis | Very frequent [Orphanet] | 158 / 7739 | |||
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(HPO:0002383) | Encephalitis | Occasional [Orphanet] | 41 / 7739 | |||
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(HPO:0100769) | Synovitis | Occasional [Orphanet] | 86 / 7739 | |||
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(HPO:0002754) | Osteomyelitis | Occasional [Orphanet] | 37 / 7739 | |||
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(HPO:0002797) | Osteolysis | Occasional [Orphanet] | 68 / 7739 | |||
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(HPO:0005059) | Arthralgia/arthritis | Very frequent [Orphanet] | 141 / 7739 | |||
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(HPO:0001396) | Cholestasis | Frequent [Orphanet] | 136 / 7739 | |||
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(HPO:0001541) | Ascites | Occasional [Orphanet] | 94 / 7739 | |||
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(HPO:0002027) | Abdominal pain | Very frequent [Orphanet] | 184 / 7739 | |||
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(HPO:0100762) | Hemobilia | Occasional [Orphanet] | 1 / 7739 | |||
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(HPO:0005244) | Gastrointestinal infarctions | Frequent [Orphanet] | 14 / 7739 | |||
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(HPO:0001733) | Pancreatitis | Occasional [Orphanet] | 46 / 7739 | |||
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(HPO:0002239) | Gastrointestinal hemorrhage | Frequent [Orphanet] | 97 / 7739 | |||
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(HPO:0012437) | Abnormal gallbladder morphology | Occasional [Orphanet] | 17 / 7739 | |||
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(HPO:0002024) | Malabsorption | Occasional [Orphanet] | 142 / 7739 | |||
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(HPO:0002242) | Abnormality of the intestine | Frequent [Orphanet] | 42 / 7739 | |||
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(HPO:0004325) | Decreased body weight | Very frequent [Orphanet] | 492 / 7739 | |||
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(HPO:0001933) | Subcutaneous hemorrhage | Very frequent [Orphanet] | 50 / 7739 | |||
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(HPO:0000988) | Skin rash | Very frequent [Orphanet] | 98 / 7739 | |||
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(HPO:0001063) | Acrocyanosis | Occasional [Orphanet] | 56 / 7739 | |||
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(HPO:0200042) | Skin ulcer | Frequent [Orphanet] | 138 / 7739 | |||
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(HPO:0001025) | Urticaria | Frequent [Orphanet] | 73 / 7739 | |||
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(HPO:0000965) | Cutis marmorata | Very frequent [Orphanet] | 46 / 7739 | |||
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(HPO:0011675) | Arrhythmia | Frequent [Orphanet] | 226 / 7739 | |||
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(HPO:0100545) | Arterial stenosis | Very frequent [Orphanet] | 22 / 7739 | |||
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(HPO:0100735) | Hypertensive crisis | Very frequent [Orphanet] | 9 / 7739 | |||
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(HPO:0002617) | Aneurysm | Very frequent [Orphanet] | 34 / 7739 | |||
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(HPO:0001638) | Cardiomyopathy | Very frequent [Orphanet] | 192 / 7739 | |||
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(HPO:0001635) | Congestive heart failure | Occasional [Orphanet] | 232 / 7739 | |||
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(HPO:0001677) | Coronary artery disease | Frequent [Orphanet] | 58 / 7739 | |||
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(HPO:0002140) | Ischemic stroke | Occasional [Orphanet] | 70 / 7739 | |||
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(HPO:0001697) | Abnormality of the pericardium | Occasional [Orphanet] | 52 / 7739 | |||
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(HPO:0012089) | Arteritis | Very frequent [Orphanet] | 40 / 7739 | |||
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(HPO:0001974) | Leukocytosis | Frequent [Orphanet] | 33 / 7739 | |||
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(HPO:0004420) | Arterial thrombosis | Occasional [Orphanet] | 20 / 7739 | |||
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(HPO:0001945) | Fever | Very frequent [Orphanet] | 218 / 7739 | |||
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(HPO:0011227) | Elevated C-reactive protein level | Very frequent [Orphanet] | 55 / 7739 | |||
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(HPO:0010741) | Edema of the lower limbs | Very frequent [Orphanet] | 34 / 7739 | |||
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(HPO:0100758) | Gangrene | Frequent [Orphanet] | 25 / 7739 | |||
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(HPO:0002099) | Asthma | Very frequent [Orphanet] | 62 / 7739 | |||
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(HPO:0002960) | Autoimmunity | Occasional [Orphanet] | 78 / 7739 | |||
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(HPO:0100614) | Myositis | Occasional [Orphanet] | 21 / 7739 | |||
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(HPO:0003326) | Myalgia | Very frequent [Orphanet] | 143 / 7739 | |||
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(HPO:0030350) | Erythematous papule | Frequent [Orphanet] | 123 / 7739 |
Associated genes:
ClinVar (via SNiPA)
| Gene symbol | Variation | Clinical significance | Reference |
|---|