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Granulomatosis with polyangiitis
1 OMIM reference -
4 associated genes
64 connected diseases
66 signs/symptoms
Disease Type of connection
Juvenile rheumatoid factor-negative polyarthritis
Oligoarticular juvenile arthritis
Chronic berylliosis
Giant cell arteritis
Pediatric systemic lupus erythematosus
Severe combined immunodeficiency due to LCK deficiency
T-B+ severe combined immunodeficiency due to CD3delta / CD3epsilon / CD3zeta
Juvenile myelomonocytic leukemia
Autosomal agammaglobulinemia
SHORT syndrome
Budd-Chiari syndrome
Essential thrombocythemia
Familial thrombocytosis
Myelofibrosis with myeloid metaplasia
Polycythemia vera
Combined immunodeficiency due to ZAP70 deficiency
Noonan syndrome-like disorder with juvenile myelomonocytic leukemia
Aneurysm - osteoarthritis syndrome
Familial thoracic aortic aneurysm and aortic dissection
Adult-onset distal myopathy due to VCP mutation
Amyotrophic lateral sclerosis
Autoimmune lymphoproliferative syndrome
Autosomal recessive systemic lupus erythematosus
Behavioral variant of frontotemporal dementia
Bilateral striopallidodentate calcinosis
Chronic myelomonocytic leukemia
Common variable immunodeficiency
Familial medullary thyroid carcinoma
Hereditary sensory and autonomic neuropathy type 4
Hereditary sensory and autonomic neuropathy type 5
Idiopathic hypereosinophilic syndrome
Inclusion body myopathy with Paget disease of bone and frontotemporal dementia
Infantile myofibromatosis
Laron syndrome
Myeloid neoplasm associated with PDGFRB rearrangement
Papillary or follicular thyroid carcinoma
Progressive non-fluent aphasia
Semantic dementia
Short stature due to partial GHR deficiency
Spastic paraplegia - Paget disease of bone
Unclassified chronic myeloproliferative disease
Wiskott-Aldrich syndrome
X-linked severe congenital neutropenia
X-linked thrombocytopenia with normal platelets
Alpha-1-antitrypsin deficiency
Autosomal dominant cutis laxa
Hemorrhagic disease due to alpha-1-antitrypsin Pittsburgh mutation
Supravalvular aortic stenosis
Williams syndrome
Acute promyelocytic leukemia
Laron syndrome with immunodeficiency
Childhood-onset nemaline myopathy
Congenital fiber-type disproportion myopathy
Congenital myopathy with excess of thin filaments
Familial vesicoureteral reflux
Hereditary spherocytosis
Intermediate nemaline myopathy
Severe congenital nemaline myopathy
Typical nemaline myopathy
LEOPARD syndrome
Metachondromatosis
Noonan syndrome
Anaplastic ependymoma
Leukocyte adhesion deficiency type I
Synonym(s):
- GPA
- Wegener granulomatosis
Classification (Orphanet):
- Rare neurologic disease
- Rare renal disease
- Rare respiratory disease
- Rare systemic or rheumatologic disease
Classification (ICD10):
- Diseases of the musculoskeletal system and connective tissue -
Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: variable
Average age of death: -
Type of inheritance: sporadic
External references:
1 OMIM reference -
1 MeSH reference: D014890
Gene symbol UniProt reference OMIM reference
CTLA4 P16410123890
HLA-DPB1 P04440142858
PRTN3 P24158177020
PTPN22 Q9Y2R2600716
Very frequent
- Anomalies of nose and olfaction
- Articular / joint pain / arthralgia
- Asthenia / fatigue / weakness
- Autoimmunity / autoimmune reaction / autoantibodies
- Enanthema / aphtosa / aphta / leukoplakia
- Epistaxis / nose bleeding
- Fever / chilling
- Ground-glass / reticular / alveolar / interstitial lung / pulmonary opacity
- Hematuria / microhematuria
- Nasal congestion / sinusitis / rhinitis / rhinorrhea
- Paresthesia / dysesthesia / hypoesthesia / anesthesia / numbness
- Renal glomerular defect / glomerulopathy
- Repeat respiratory infections
- Transient cerebral ischemia / stroke
- Vascularitis / vasculitides / arteritis
- Weight loss / loss of appetite / break in weight curve / general health alteration

Frequent
- Acute abdominal pain / colic
- Biological inflammatory syndrome / increased erythrocyte sedimentation rate / CRP
- Chronic obstructive pulmonary disease / COPD / obstructive respiratory syndrome
- Chronic uveitis / blepharitis / episcleritis / scleritis / conjonctivitis / keratitis
- Cough
- Cutaneous rash
- Follicular / erythematous / edematous papules / milium
- Hemoptysis
- Hypothalamic-hypophyseal axis anomalies / hypothalamus / pituitary anomalies
- Lung fibrosis
- Lung / pulmonary infiltrates
- Lung / pulmonary / alveolar hemorrhage / hematoma
- Nausea / vomiting / regurgitation / merycism / hyperemesis
- Neuritis / polyneuritis / multineuritis
- Palpebral edema / periorbital edema
- Peripheral neuropathy
- Proteinuria
- Respiratory distress / dyspnea / respiratory failure / lung volume reduction
- Thoracic / chest pain

Occasional
- Abnormal pleura / hydrothorax / pleuresia / pleural effusion / chylothorax
- Angor pectoris / myocardial infarction
- Cardiac rhythm disorder / arrhythmia
- Chronic arterial hypertension
- Chronic skin infection / ulcerations / ulcers / cancrum
- Chronic / relapsing otitis
- Cranial nerves palsy
- Diabetes insipidus
- Facial pain / cephalalgia / migraine
- Gangrena / necrosis
- Gastrointestinal bleeding / hemorrhage / hematemesis / melena / rectorrhagia
- Hemiplegia / diplegia / hemiparesia / limb palsy
- Interstitial / restrictive pneumopathy / restrictive respiratory syndrome
- Intestinal obstruction / ileus
- Intestinal perforation
- Megaureter / hydronephrosis / pyeloureteral junction syndrome
- Meningitis / meningeal syndrome
- Myalgia / muscular pain
- Pancreatitis
- Pericardium anomalies / pericarditis / absence / cysts / pericardial effusion
- Proptosis / exophthalmos
- Prostatic inflammation / prostatitis
- Purpura / petichiae
- Renal failure
- Retinopathy
- Seizures / epilepsy / absences / spasms / status epilepticus
- Sensitive trouble / deficit
- Sensorineural deafness / hearing loss
- Ureteral stenosis / narrowing
- Venous thrombosis / phlebitis / thrombophlebitis
- Visual loss / blindness / amblyopia