Granulomatosis with polyangiitis

General Information (adopted from Orphanet):

Synonyms, Signs: GRANULOMATOSIS WITH POLYANGITIS
GPA
WG
Wegener granulomatosis
Number of Symptoms 65
OrphanetNr: 900
OMIM Id: 608710
ICD-10: M31.3
UMLs: C0043092
MeSH: D014890
MedDRA: 10047888
Snomed: 195353004
239934006

Prevalence, inheritance and age of onset:

Prevalence: 10 of 100 000 [Orphanet]
Inheritance: Not applicable
[Orphanet]
Age of onset: All ages
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Anti-neutrophil cytoplasmic antibody-associated vasculitis
 -Rare circulatory system disease
 -Rare respiratory disease
 -Rare systemic or rheumatologic disease
Inflammatory and autoimmune disease with epilepsy
 -Rare neurologic 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:0000093) Proteinuria Frequent [Orphanet] 169 / 7739
2
 (HPO:0000790) Hematuria Very frequent [Orphanet] 106 / 7739
3
 (HPO:0000072) Hydroureter Occasional [Orphanet] 146 / 7739
4
 (HPO:0000024) Prostatitis Occasional [Orphanet] 4 / 7739
5
 (HPO:0100820) Glomerulopathy Very frequent [Orphanet] 46 / 7739
6
 (HPO:0000083) Renal insufficiency Occasional [Orphanet] 232 / 7739
7
 (HPO:0000071) Ureteral stenosis Occasional [Orphanet] 9 / 7739
8
 (HPO:0000606) Abnormality of the periorbital region Frequent [Orphanet] 96 / 7739
9
 (HPO:0000520) Proptosis Occasional [Orphanet] 192 / 7739
10
 (HPO:0000366) Abnormality of the nose Very frequent [Orphanet] 56 / 7739
11
 (HPO:0000246) Sinusitis Very frequent [Orphanet] 73 / 7739
12
 (HPO:0011830) Abnormality of oral mucosa Very frequent [Orphanet] 47 / 7739
13
 (HPO:0000421) Epistaxis Very frequent [Orphanet] 85 / 7739
14
 (HPO:0000572) Visual loss Occasional [Orphanet] 272 / 7739
15
 (HPO:0100533) Inflammatory abnormality of the eye Frequent [Orphanet] 70 / 7739
16
 (HPO:0000488) Retinopathy Occasional [Orphanet] 75 / 7739
17
 (HPO:0000407) Sensorineural hearing impairment Occasional [Orphanet] 524 / 7739
18
 (HPO:0000389) Chronic otitis media Occasional [Orphanet] 64 / 7739
19
 (HPO:0004374) Hemiplegia/hemiparesis Occasional [Orphanet] 158 / 7739
20
 (HPO:0002315) Headache Occasional [Orphanet] 175 / 7739
21
 (HPO:0000763) Sensory neuropathy Very frequent [Orphanet] 78 / 7739
22
 (HPO:0001250) Seizures Occasional [Orphanet] 1245 / 7739
23
 (HPO:0009830) Peripheral neuropathy Frequent [Orphanet] 206 / 7739
24
 (HPO:0003474) Sensory impairment Very frequent [Orphanet] 54 / 7739
25
 (HPO:0006824) Cranial nerve paralysis Occasional [Orphanet] 81 / 7739
26
 (HPO:0001287) Meningitis Occasional [Orphanet] 46 / 7739
27
 (HPO:0000873) Diabetes insipidus Occasional [Orphanet] 34 / 7739
28
 (HPO:0000864) Abnormality of the hypothalamus-pituitary axis Frequent [Orphanet] 23 / 7739
29
 (HPO:0005059) Arthralgia/arthritis Very frequent [Orphanet] 141 / 7739
30
 (HPO:0100749) Chest pain Frequent [Orphanet] 92 / 7739
31
 (HPO:0002017) Nausea and vomiting Frequent [Orphanet] 134 / 7739
32
 (HPO:0002242) Abnormality of the intestine Occasional [Orphanet] 42 / 7739
33
 (HPO:0002239) Gastrointestinal hemorrhage Occasional [Orphanet] 97 / 7739
34
 (HPO:0005214) Intestinal obstruction Occasional [Orphanet] 35 / 7739
35
 (HPO:0001733) Pancreatitis Occasional [Orphanet] 46 / 7739
36
 (HPO:0002027) Abdominal pain Frequent [Orphanet] 184 / 7739
37
 (HPO:0004325) Decreased body weight Very frequent [Orphanet] 492 / 7739
38
 (HPO:0000988) Skin rash Frequent [Orphanet] 98 / 7739
39
 (HPO:0001933) Subcutaneous hemorrhage Occasional [Orphanet] 50 / 7739
40
 (HPO:0200042) Skin ulcer Occasional [Orphanet] 138 / 7739
41
 (HPO:0001677) Coronary artery disease Occasional [Orphanet] 58 / 7739
42
 (HPO:0002140) Ischemic stroke Very frequent [Orphanet] 70 / 7739
43
 (HPO:0000822) Hypertension Occasional [Orphanet] 224 / 7739
44
 (HPO:0001697) Abnormality of the pericardium Occasional [Orphanet] 52 / 7739
45
 (HPO:0012089) Arteritis Very frequent [Orphanet] 40 / 7739
46
 (HPO:0011675) Arrhythmia Occasional [Orphanet] 226 / 7739
47
 (HPO:0002955) Granulomatosis 6 / 7739
48
 (HPO:0004936) Venous thrombosis Occasional [Orphanet] 41 / 7739
49
 (HPO:0011227) Elevated C-reactive protein level Frequent [Orphanet] 55 / 7739
50
 (HPO:0001945) Fever Very frequent [Orphanet] 218 / 7739
51
 (HPO:0100758) Gangrene Occasional [Orphanet] 25 / 7739
52
 (HPO:0006510) Chronic obstructive pulmonary disease Frequent [Orphanet] 19 / 7739
53
 (HPO:0002206) Pulmonary fibrosis Frequent [Orphanet] 51 / 7739
54
 (HPO:0002103) Abnormality of the pleura Occasional [Orphanet] 58 / 7739
55
 (HPO:0002113) Pulmonary infiltrates Frequent [Orphanet] 36 / 7739
56
 (HPO:0002093) Respiratory insufficiency Frequent [Orphanet] 410 / 7739
57
 (HPO:0002091) Restrictive ventilatory defect Occasional [Orphanet] 46 / 7739
58
 (HPO:0002105) Hemoptysis Frequent [Orphanet] 30 / 7739
59
 (HPO:0002205) Recurrent respiratory infections Very frequent [Orphanet] 254 / 7739
60
 (HPO:0002088) Abnormality of lung morphology Frequent [Orphanet] 11 / 7739
61
 (HPO:0002960) Autoimmunity Very frequent [Orphanet] 78 / 7739
62
 (HPO:0003326) Myalgia Occasional [Orphanet] 143 / 7739
63
 (HPO:0001324) Muscle weakness Very frequent [Orphanet] 859 / 7739
64
 (HPO:0030350) Erythematous papule Frequent [Orphanet] 123 / 7739
65
 ([DEL]MedDRA:10011224) Cough Frequent [Orphanet] 70 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Wegener granulomatosis (WG) is a systemic disease with a complex genetic background. It is characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis, vasculitis, and the presence of antineutrophil cytoplasmatic autoantibodies (ANCAs) in patient ...