GPA
MCID: WGN002
MIFTS: 71

Wegener's Granulomatosis (GPA) malady

Neuronal diseases, Nephrological diseases, Respiratory diseases, Bone diseases, Rare diseases, Immune diseases, Cardiovascular diseases, Skin diseases categories
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Summaries for Wegener's Granulomatosis

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8Disease Ontology, 47OMIM, 33MalaCards
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Disease Ontology:8 An autoimmune disease that is a vasculitis that is located in lung, located in kidney, located in skin resulting from an autoimmune attack by antineutrophil cytoplasmic antibodies against small and medium-size blood vessels.

MalaCards: Wegener's Granulomatosis, also known as wegener granulomatosis, is related to polyarteritis nodosa and churg-strauss syndrome, and has symptoms including vascularitis/vasculitides/arteritis, lung/pulmonary/alveolar hemorrhage/hematoma and meningitis/meningeal syndrome. An important gene associated with Wegener's Granulomatosis is WG (Wegener granulomatosis), and among its related pathways are CD28 co-stimulation and Cell adhesion Cell matrix glycoconjugates. The drug cyclophosphamide and the compounds propylthiouracil and gliclazide have been mentioned in the context of this disorder. Affiliated tissues include kidney, lung and skin, and related mouse phenotypes are liver/biliary system and hematopoietic system.

Description from OMIM:47 608710

Aliases & Classifications for Wegener's Granulomatosis

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8Disease Ontology, 9diseasecard, 47OMIM, 49Orphanet, 62UMLS, 10DISEASES, 45Novoseek, 27ICD9CM, 58SNOMED-CT, 35MeSH, 40NCIt, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet, 25ICD10
See all sources

Classifications:



Characteristics (Orphanet epidemiological data):

49
wegener granulomatosis:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Variable


Aliases & Descriptions:

wegener's granulomatosis 8
wegener granulomatosis 8 9 47 49 62
wegeners granulomatosis 10 45
necrotizing respiratory granulomatosis 8
granulomatosis with polyangiitis 49
granulomatosis - wegener's 8
wegener's syndrome 8
gpa 49


External Ids:

Disease Ontology8 DOID:12132
OMIM47 608710
ICD9CM27 446.4
MeSH35 D014890
NCIt40 C3444
MESH via Orphanet36 D014890
ICD10 via Orphanet26 M31.3
SNOMED-CT via Orphanet59 195353004, 239934006
UMLS via Orphanet63 C0043092
ICD1025 M31.3

Related Diseases for Wegener's Granulomatosis

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17GeneCards, 18GeneDecks
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Diseases in the Wegener's Granulomatosis family:

Wegener-Like Granulomatosis

Diseases related to Wegener's Granulomatosis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 116)
idRelated DiseaseScoreTop Affiliating Genes
1polyarteritis nodosa30.8PRTN3, MPO
2churg-strauss syndrome30.7PRTN3, MPO
3vasculitis30.6SULT1A3, CTLA4, MPO, SELE, PRTN3
4ulcerative colitis30.4TNFRSF4, MPO, PRTN3, CTLA4, SELE
5mononeuritis multiplex30.3PRTN3, MPO
6rapidly progressive glomerulonephritis30.3PRTN3, MPO
7systemic lupus erythematosus30.2CCR4, TNFRSF4, PTPN22, MPO, SELE, SULT1A3
8arthritis30.1PTPN22, CTLA4, CXCL6, MPO, SULT1A3, PRTN3
9thyroiditis30.1PTPN22, CTLA4
10rheumatoid arthritis29.9PTPN22, SELE, MPO, HLA-DPB1, TNFRSF4, TNFRSF18
11eosinophilic granulomatosis with polyangiitis10.9
12neuropathy10.6
13granulomatosis with polyangiitis10.6
14microscopic polyangiitis10.4
15aneurysm10.4
16cytomegalovirus infection10.4
17purpura10.3
18bronchiolitis10.2
19bronchiolitis obliterans10.2
20bronchiolitis obliterans organizing pneumonia10.2
21sialadenitis10.2
22subacute bacterial endocarditis10.2
23idiopathic pulmonary fibrosis10.2
24hydronephrosis10.2
25aortic aneurysm10.2
26endocarditis10.2
27intestinal perforation10.2
28lung cancer10.2
29lupus erythematosus10.2
30periostitis10.2
31peripheral neuropathy10.2
32pneumonia10.2
33pulmonary fibrosis10.2
34sleep disorder10.2
35t cell deficiency10.2
36tuberculosis10.2
37allergic angiitis10.2
38coronary artery aneurysm10.2
39hypereosinophilic syndrome10.2
40thromboembolism10.2
41fibromyalgia10.2
42endotheliitis10.2
43pustulosis palmaris et plantaris10.1SELE
44hypersensitivity vasculitis10.1MPO, PRTN3
45mononeuropathy10.1MPO, PRTN3
46panhypopituitarism10.1
47cicatricial pemphigoid10.1
48goodpasture syndrome10.1
49pyoderma gangrenosum10.1
50choroiditis10.1

Graphical network of the top 20 diseases related to Wegener's Granulomatosis:



Diseases related to wegener's granulomatosis

Symptoms for Wegener's Granulomatosis

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Sources:
47OMIM, 49Orphanet
See all sources


Clinical features from OMIM:

608710

Symptoms:

49 (show all 66)
  • vascularitis/vasculitides/arteritis
  • lung/pulmonary/alveolar hemorrhage/hematoma
  • meningitis/meningeal syndrome
  • ground-glass/reticular/alveolar/interstitial lung/pulmonary opacity
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • epistaxis/nose bleeding
  • biological inflammatory syndrome/increased erythrocyte sedimentation rate/crp
  • megaureter/hydronephrosis/pyeloureteral junction syndrome
  • hypothalamic-hypophyseal axis anomalies/hypothalamus/pituitary anomalies
  • chronic obstructive pulmonary disease/copd/obstructive respiratory syndrome
  • palpebral edema/periorbital edema
  • proptosis/exophthalmos
  • hemoptysis
  • ureteral stenosis/narrowing
  • neuritis/polyneuritis/multineuritis
  • intestinal perforation
  • prostatic inflammation/prostatitis
  • anomalies of nose and olfaction
  • enanthema/aphtosa/aphta/leukoplakia
  • asthenia/fatigue/weakness
  • paresthesia/dysesthesia/hypoesthesia/anesthesia/numbness
  • nausea/vomiting/regurgitation/merycism/hyperemesis
  • renal failure
  • cranial nerves palsy
  • fever/chilling
  • articular/joint pain/arthralgia
  • lung fibrosis
  • cardiac rhythm disorder/arrhythmia
  • chronic skin infection/ulcerations/ulcers/cancrum
  • cutaneous rash
  • follicular/erythematous/edematous papules/milium
  • retinopathy
  • diabetes insipidus
  • seizures/epilepsy/absences/spasms/status epilepticus
  • hemiplegia/diplegia/hemiparesia/limb palsy
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • chronic arterial hypertension
  • chronic/relapsing otitis
  • repeat respiratory infections
  • sensorineural deafness/hearing loss
  • visual loss/blindness/amblyopia
  • proteinuria
  • peripheral neuropathy
  • sensitive trouble/deficit
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • lung/pulmonary infiltrates
  • hematuria/microhematuria
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • venous thrombosis/phlebitis/thrombophlebitis
  • facial pain/cephalalgia/migraine
  • transient cerebral ischemia/stroke
  • pancreatitis
  • purpura/petichiae
  • intestinal obstruction/ileus
  • gastrointestinal bleeding/hemorrhage/hematemesis/melena/rectorrhagia
  • angor pectoris/myocardial infarction
  • nasal congestion/sinusitis/rhinitis/rhinorrhea
  • thoracic/chest pain
  • cough
  • interstitial/restrictive pneumopathy/restrictive respiratory syndrome
  • gangrena/necrosis
  • myalgia/muscular pain
  • autoimmunity/autoimmune reaction/autoantibodies
  • acute abdominal pain/colic
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • renal glomerular defect/glomerulopathy

Drugs & Therapeutics for Wegener's Granulomatosis

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Wegener's Granulomatosis

Search NIH Clinical Center for Wegener's Granulomatosis

Inferred drug relations via UMLS62/NDF-RT41:

Genetic Tests for Wegener's Granulomatosis

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Anatomical Context for Wegener's Granulomatosis

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Sources:
33MalaCards, 14FMA
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MalaCards organs/tissues related to Wegener's Granulomatosis:

33
Lung, Skin, Kidney, T cells, Prostate, Endothelial, Pituitary, Bone, B cells, Hypothalamus, Thyroid, Testes, Colon, Heart, Salivary gland, Breast, Eye, Small intestine, Brain, Tongue, Monocytes, Occipital lobe, Spinal cord

FMA organs/tissues related to Wegener's Granulomatosis:

14
Kidney, Lung, Skin

Animal Models for Wegener's Granulomatosis or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Wegener's Granulomatosis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053709.3PTPN22, SELE, XCL1, CTLA4, TNFRSF4
2MP:00053977.3CCR4, XCL1, MS4A2, CXCL6, PRTN3, SELE
3MP:00053877.1CTLA4, TNFRSF4, XCL1, CCR4, MS4A2, CXCL6

Publications for Wegener's Granulomatosis

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Sources:
52PubMed
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Articles related to Wegener's Granulomatosis:

(show top 50)    (show all 764)
idTitleAuthorsYear
1
Treatment of orbital inflammation with rituximab in Wegener's granulomatosis. (22272561)
2012
2
Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis--a clinical randomized controlled trial. (20577017)
2011
3
Wegener's granulomatosis of the orbit: a review of clinical features and updates in diagnosis and treatment. (21958186)
2011
4
Clinical images: Reticular rash in a patient with Wegener's granulomatosis. (21360516)
2011
5
Outcomes of renal transplantation in recipients with Wegener's granulomatosis. (20394635)
2011
6
Wegener's granulomatosis with multiple pulmonary nodules - diagnostic difficulties. (21103126)
2010
7
Refractory otitis media as the first manifestation of Wegener's granulomatosis. (20835547)
2010
8
Chemosensory function in Wegener's granulomatosis: a preliminary report. (20043173)
2010
9
Survival of patients with Wegener's granulomatosis diagnosed in Finland in 1981-2000. (20132074)
2010
10
Pulmonary alveolar proteinosis in a 67-year-old woman with Wegener's granulomatosis. (20623200)
2010
11
Unexpectedly difficult intubation caused by subglottic stenosis in Wegener's granulomatosis. (20094734)
2010
12
Surgical interventions and local therapy for Wegener's granulomatosis. (19910793)
2010
13
Successful treatment of hypertrophic pachymeningitis in refractory Wegener's granulomatosis with rituximab. (19802640)
2010
14
Effectiveness of rituximab in severe Wegener's granulomatosis: report of two cases and review of the literature. (19590594)
2009
15
Generalized Wegener's granulomatosis in an immunocompetent adult after cytomegalovirus mononucleosis and bacterial urinary tract infection. (19404959)
2009
16
Expression of CD57 on CD8+ T lymphocytes of patients with Wegener's granulomatosis and microscopic polyangiitis: evidence for continuous activation of CD8+ cells. (19646341)
2009
17
Cardiomyopathy associated with Wegener's granulomatosis. (21687156)
2009
18
Wegener's granulomatosis. (19266823)
2009
19
Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease. (19175952)
2009
20
Wegener's granulomatosis patients show an adequate antibody response to influenza vaccination. (18625625)
2009
21
Subglottic stenosis as a complication of Wegener's granulomatosis. (18253680)
2008
22
A novel system to test for specificity of B cell receptors from tissue of Wegener's granulomatosis patients. (18799062)
2008
23
Does the dose of methotrexate influence the rate of relapse in patients with Wegener's granulomatosis? (17998931)
2008
24
Balancing efficacy and cancer risk of cyclophosphamide for patients with Wegener's granulomatosis. (18506161)
2008
25
Can sulfasalazine therapy induce or exacerbate Wegener's granulomatosis? (18189198)
2008
26
The p53 G72C and MDM2 T309G single nucleotide polymorphisms in patients with Wegener's granulomatosis. (18799058)
2008
27
Acute interstitial nephritis complicating a new diagnosis of Wegener's granulomatosis. (18309834)
2008
28
Wegener's granulomatosis mimicking facial granulomatous rosacea: a separate subset? (17989900)
2007
29
Wegener's granulomatosis presenting with granulomatous facial erythematous papules and plaques mimicking granulomatous rosacea. (16792787)
2006
30
Microscopic polyangiitis or Wegener's granulomatosis? That is the question. (16548912)
2006
31
Wegener's granulomatosis presenting as diabetes insipidus. (17115352)
2006
32
Wegener's granulomatosis presenting with diffuse alveolar hemorrhage and negative antineutrophilic cytoplasmic antibody test. (16933823)
2006
33
Intestinal perforation and jejunal haemorrhage due to Wegener's granulomatosis. (15789904)
2005
34
Activation, apoptosis, and clearance of neutrophils in Wegener's granulomatosis. (16126939)
2005
35
Orbital socket contracture: a complication of inflammatory orbital disease in patients with Wegener's granulomatosis. (15774931)
2005
36
Aneurysmal subarachnoid hemorrhage in a patient with Wegener's granulomatosis. (15480908)
2004
37
Orbital inflammatory pseudotumor due to hypersensitivity vasculitis and mononeuritis multiplex in a patient with atypical, cANCA-positive Wegener's granulomatosis. (12700924)
2003
38
Wegener's granulomatosis presenting as acute suppurative interstitial nephritis. (11577128)
2001
39
Facial paralysis in Wegener's granulomatosis of the middle ear. (11276335)
2001
40
In vitro up-regulation of E-selectin and induction of interleukin-6 in endothelial cells by autoantibodies in Wegener's granulomatosis and microscopic polyangiitis. (10464553)
1999
41
Antineutrophil cytoplasmic antibodies in Wegener's granulomatosis. (9875021)
1998
42
Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease. (9355084)
1997
43
The phylogeny of proteinase 3/myeloblastin, the autoantigen in Wegener's granulomatosis, and myeloperoxidase as shown by immunohistochemical studies on human leukemic cell lines. (8261669)
1994
44
A diagnostic dilemma: Wegener's granulomatosis, relapsing polychondritis or both? (8258052)
1993
45
Structure, chromosomal assignment, and expression of the gene for proteinase-3. The Wegener's granulomatosis autoantigen. (1400430)
1992
46
Distribution pattern of proteinase 3 in Wegener's granulomatosis and other vasculitic diseases]. (2060965)
1991
47
Scleritis and Wegener's granulomatosis in children. (1882959)
1991
48
Anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis recognize an elastinolytic enzyme. (1688612)
1990
49
Small intestinal perforation in Wegener's granulomatosis. (7100026)
1982
50
Subacute meningitis heralding a diffuse granulomatous angiitis: (Wegener's granulomatosis?). (557762)
1977

Variations for Wegener's Granulomatosis

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Expression for genes affiliated with Wegener's Granulomatosis

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Sources:
2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Wegener's Granulomatosis

Search GEO for disease gene expression data for Wegener's Granulomatosis.

Pathways for genes affiliated with Wegener's Granulomatosis

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Sources:
50PathCards, 55Reactome, 53QIAGEN, 60Thomson Reuters, 30KEGG, 38NCBI BioSystems Database, 57SinoBiological, 54R&D Systems
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Pathways related to Wegener's Granulomatosis according to GeneCards/GeneDecks:

(show all 14)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.9HLA-DPB1, PTPN22, CTLA4
29.9XCL1, SELE
39.9SELE, HLA-DPB1, CTLA4
4
Show member pathways
9.9HLA-DPB1, MS4A2, CTLA4
59.8HLA-DPB1, CTLA4, CXCL6
6
Show member pathways
9.5TNFRSF4, TNFRSF18, TNFSF9
7
Show member pathways
9.3TNFSF9, TNFRSF18, TNFRSF4, HLA-DPB1
8
Show member pathways
9.2CCR4, XCL1, CCL21, CXCL6
9
Show member pathways
9.2CCR4, XCL1, CXCL6, CCL21
10
Show member pathways
9.2CXCL6, CCL21, CCR4, XCL1
11
Show member pathways
8.6TNFRSF4, CCL21, CCR4, CXCL6, TNFRSF18, TNFSF9
12
Show member pathways
8.6CCR4, CXCL6, TNFSF9, TNFRSF4, TNFRSF18, CCL21
13
Show member pathways
8.4CXCL6, TNFSF9, TNFRSF18, TNFRSF4, CCR4, CCL21
14
Show member pathways
8.2TNFRSF4, XCL1, CCL21, TNFRSF18, CCR4, CXCL6

Compounds for genes affiliated with Wegener's Granulomatosis

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Sources:
45Novoseek, 3BitterDB, 11DrugBank, 51PharmGKB, 24HMDB, 29IUPHAR, 61Tocris Bioscience
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Compounds related to Wegener's Granulomatosis according to GeneCards/GeneDecks:

(show all 17)
idCompoundScoreTop Affiliating Genes
1propylthiouracil45 3 1112.4MPO, PRTN3
2gliclazide45 51 1112.3SELE, MPO
3infliximab45 51 1112.2SELE, CTLA4, PRTN3
4teck4510.1CCR4, CCL21
5ionomycin4510.1TNFRSF4, PRTN3, CTLA4
6formaldehyde45 2411.1SELE, MPO, PRTN3
7larc4510.1CCR4, CCL21
8groalpha4510.0CXCL6, SELE
9ccl345 2910.8CCL21, XCL1, CCR4
10dmso459.8PRTN3, SELE, MPO
11ccl445 2910.8CCL21, XCL1, CCR4
12apocynin45 6110.7MPO, SELE
13heparin45 29 24 1112.5XCL1, SULT1A3, MPO, SELE
14creatinine459.5MPO, IL18BP, SULT1A3, SELE
15rantes459.1SELE, CXCL6, CCR4, CCL21, XCL1, CTLA4
16serine459.1CXCL6, CTLA4, PRTN3, SELE, SERPINA2, MPO
17calcium45 51 24 1111.8CCR4, CTLA4, XCL1, CCL21, CXCL6, PRTN3

GO Terms for genes affiliated with Wegener's Granulomatosis

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Sources:
16Gene Ontology
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Cellular components related to Wegener's Granulomatosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:0098979.1TNFRSF4, TNFRSF18, MS4A2, CCR4, CTLA4
2integral component of plasma membraneGO:0058878.8TNFRSF4, TNFRSF18, MS4A2, CCR4, CTLA4
3plasma membraneGO:0058868.7TNFSF9, SELE, PRTN3, MS4A2, CCR4, CD177
4extracellular regionGO:0055768.2TNFRSF18, SERPINA2, CXCL6, CCL21, IL18BP, XCL1
5extracellular spaceGO:0056158.1XCL1, TNFSF9, MPO, SERPINA2, SELE, CXCL6

Biological processes related to Wegener's Granulomatosis according to GeneCards/GeneDecks:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1release of sequestered calcium ion into cytosolGO:0512099.8XCL1, CCL21
2T cell costimulationGO:0312959.8CTLA4, CCL21, HLA-DPB1
3response to lipopolysaccharideGO:0324969.5MPO, SELE, IL18BP
4positive regulation of neutrophil chemotaxisGO:0900239.5XCL1, CCL21
5cell-cell signalingGO:0072679.2TNFSF9, CXCL6, CCL21, XCL1
6inflammatory responseGO:0069549.0TNFRSF4, SELE, CXCL6, MS4A2, CCR4, CCL21
7immune responseGO:0069558.4CTLA4, TNFRSF4, TNFSF9, CXCL6, MS4A2, CCR4

Molecular functions related to Wegener's Granulomatosis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1tumor necrosis factor-activated receptor activityGO:0050319.8TNFRSF18, TNFRSF4
2chemokine receptor bindingGO:0423799.5XCL1, CCL21
3chemokine activityGO:0080099.4XCL1, CCL21, CXCL6

Products for genes affiliated with Wegener's Granulomatosis

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  • Antibodies
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Sources for Wegener's Granulomatosis

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet