Polyarteritis nodosa

General Information (adopted from Orphanet):

Synonyms, Signs: Periarteritis nodosa
K├╝ssmaul-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: Sort by abundance 

1
(HPO:0000112) Nephropathy Very frequent [Orphanet] 92 / 7739
2
(HPO:0000071) Ureteral stenosis Occasional [Orphanet] 9 / 7739
3
(HPO:0000083) Renal insufficiency Very frequent [Orphanet] 232 / 7739
4
(HPO:0100796) Orchitis Very frequent [Orphanet] 7 / 7739
5
(HPO:0008046) Abnormality of the retinal vasculature Occasional [Orphanet] 41 / 7739
6
(HPO:0100533) Inflammatory abnormality of the eye Occasional [Orphanet] 70 / 7739
7
(HPO:0000541) Retinal detachment Occasional [Orphanet] 87 / 7739
8
(HPO:0002071) Abnormality of extrapyramidal motor function Occasional [Orphanet] 76 / 7739
9
(HPO:0007256) Abnormal pyramidal signs Very frequent [Orphanet] 116 / 7739
10
(HPO:0000763) Sensory neuropathy Very frequent [Orphanet] 78 / 7739
11
(HPO:0000708) Behavioral abnormality Frequent [Orphanet] 212 / 7739
12
(HPO:0002315) Headache Very frequent [Orphanet] 175 / 7739
13
(HPO:0001250) Seizures Frequent [Orphanet] 1245 / 7739
14
(HPO:0009830) Peripheral neuropathy Very frequent [Orphanet] 206 / 7739
15
(HPO:0004374) Hemiplegia/hemiparesis Very frequent [Orphanet] 158 / 7739
16
(HPO:0002383) Encephalitis Occasional [Orphanet] 41 / 7739
17
(HPO:0100769) Synovitis Occasional [Orphanet] 86 / 7739
18
(HPO:0002754) Osteomyelitis Occasional [Orphanet] 37 / 7739
19
(HPO:0002797) Osteolysis Occasional [Orphanet] 68 / 7739
20
(HPO:0005059) Arthralgia/arthritis Very frequent [Orphanet] 141 / 7739
21
(HPO:0001396) Cholestasis Frequent [Orphanet] 136 / 7739
22
(HPO:0001541) Ascites Occasional [Orphanet] 94 / 7739
23
(HPO:0002027) Abdominal pain Very frequent [Orphanet] 184 / 7739
24
(HPO:0100762) Hemobilia Occasional [Orphanet] 1 / 7739
25
(HPO:0005244) Gastrointestinal infarctions Frequent [Orphanet] 14 / 7739
26
(HPO:0001733) Pancreatitis Occasional [Orphanet] 46 / 7739
27
(HPO:0002239) Gastrointestinal hemorrhage Frequent [Orphanet] 97 / 7739
28
(HPO:0012437) Abnormal gallbladder morphology Occasional [Orphanet] 17 / 7739
29
(HPO:0002024) Malabsorption Occasional [Orphanet] 142 / 7739
30
(HPO:0002242) Abnormality of the intestine Frequent [Orphanet] 42 / 7739
31
(HPO:0004325) Decreased body weight Very frequent [Orphanet] 492 / 7739
32
(HPO:0001933) Subcutaneous hemorrhage Very frequent [Orphanet] 50 / 7739
33
(HPO:0000988) Skin rash Very frequent [Orphanet] 98 / 7739
34
(HPO:0001063) Acrocyanosis Occasional [Orphanet] 56 / 7739
35
(HPO:0200042) Skin ulcer Frequent [Orphanet] 138 / 7739
36
(HPO:0001025) Urticaria Frequent [Orphanet] 73 / 7739
37
(HPO:0000965) Cutis marmorata Very frequent [Orphanet] 46 / 7739
38
(HPO:0011675) Arrhythmia Frequent [Orphanet] 226 / 7739
39
(HPO:0100545) Arterial stenosis Very frequent [Orphanet] 22 / 7739
40
(HPO:0100735) Hypertensive crisis Very frequent [Orphanet] 9 / 7739
41
(HPO:0002617) Aneurysm Very frequent [Orphanet] 34 / 7739
42
(HPO:0001638) Cardiomyopathy Very frequent [Orphanet] 192 / 7739
43
(HPO:0001635) Congestive heart failure Occasional [Orphanet] 232 / 7739
44
(HPO:0001677) Coronary artery disease Frequent [Orphanet] 58 / 7739
45
(HPO:0002140) Ischemic stroke Occasional [Orphanet] 70 / 7739
46
(HPO:0001697) Abnormality of the pericardium Occasional [Orphanet] 52 / 7739
47
(HPO:0012089) Arteritis Very frequent [Orphanet] 40 / 7739
48
(HPO:0001974) Leukocytosis Frequent [Orphanet] 33 / 7739
49
(HPO:0004420) Arterial thrombosis Occasional [Orphanet] 20 / 7739
50
(HPO:0001945) Fever Very frequent [Orphanet] 218 / 7739
51
(HPO:0011227) Elevated C-reactive protein level Very frequent [Orphanet] 55 / 7739
52
(HPO:0010741) Edema of the lower limbs Very frequent [Orphanet] 34 / 7739
53
(HPO:0100758) Gangrene Frequent [Orphanet] 25 / 7739
54
(HPO:0002099) Asthma Very frequent [Orphanet] 62 / 7739
55
(HPO:0002960) Autoimmunity Occasional [Orphanet] 78 / 7739
56
(HPO:0100614) Myositis Occasional [Orphanet] 21 / 7739
57
(HPO:0003326) Myalgia Very frequent [Orphanet] 143 / 7739
58
(HPO:0030350) Erythematous papule Frequent [Orphanet] 123 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: