Granulomatosis with polyangiitis

Preferred Label : Granulomatosis with polyangiitis;

Symbol : GPA;

CISMeF acronym : GPA; WG;

Type : Phenotype or locus, molecular basis unknown;

Alternative titles and symbols : WG; Wegener granulomatosis;

Description : 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 sera. These ANCAs are antibodies to a defined target antigen, proteinase-3 (PR3, PRTN3; 177020), that is present within primary azurophil granules of neutrophils (PMNs) and lysozymes of monocytes. On cytokine priming of PMNs, PR3 translocates to the cell surface, where PR3-ANCAs can interact with their antigens and activate PMNs. PMNs from patients with active WG express PR3 on their surface, produce respiratory burst, and release proteolytic enzymes after activation with PR3-ANCAs. The consequence is a self-sustaining inflammatory process (Jagiello et al., 2004).;

Prefixed ID : %608710;

Details

Origin ID

608710;

UMLS CUI

C3495801;

Automatic exact mappings (from CISMeF team)
- GYPA wt Allele [NCIt concept]
- Gestational Carriers [MeSH Descriptor]
- Graded Prognostic Assessment [NCIt concept]
- Pauci-Immune Glomerulonephritis associated with Granulomatosis with Polyangiitis [NCIt concept]
- glycophorin A (MNS blood group) [HGNC gene]
Currated CISMeF NLP mapping
- Granulomatosis with Polyangiitis [MeSH Descriptor]
- Granulomatosis with Polyangiitis [MeSH concept]
- Granulomatosis with Polyangiitis [NCIt concept]
- Granulomatosis with polyangiitis [ICD-11 Sub-sub category]
- Granulomatosis with polyangiitis [MedDRA Preferred Term]
- Granulomatosis with polyangiitis [Radlex concept]
- Granulomatosis with polyangiitis [ORDO Disease]
- Granulomatosis with polyangiitis (disorder) [SNOMED CT concept]
- Granulomatous polyangiitis [MedDRA LLT]
- WEGENER'S GRANULOMATOSIS [WHO-ART Preferred Term]
- Wegener granulomatosis [PASCAL descriptor]
- Wegener granulomatosis [Disease (Nov.)]
- Wegener granulomatosis [ACR pathology]
- Wegener granulomatosis [ACR pathology]
- Wegener's granulomatosis [ICD-10 Sub-category]
- Wegener's granulomatosis [ICD-9 code]
- Wegener's granulomatosis [MedDRA LLT]
- Wegeners granulomatosis [ICD-10 Sub-category (who)]
- Wegeners granulomatosis [MedDRA LLT]
- granulomatosis with polyangiitis [DO Concept]
- granulomatosis with polyangiitis [MedlinePlus Topic]
- wegener's granulomatosis [SNOMED Notion]
DO Cross reference
- granulomatosis with polyangiitis [DO Concept]
False automatic mappings
- Glycophorin-A [NCIt concept]
HPO term(s)
- Asthenia [HPO term]
- Chronic otitis media [HPO term]
- Concave nasal ridge [HPO term]
- Conjunctivitis [HPO term]
- Cough [HPO term]
- Cytoplasmic antineutrophil antibody positivity [HPO term]
- Diffuse alveolar hemorrhage [HPO term]
- Elevated bronchoalveolar lavage fluid neutrophil proportion [HPO term]
- Episcleritis [HPO term]
- Fatigue [HPO term]
- Fever [HPO term]
- Gangrene [HPO term]
- Granulomatosis [HPO term]
- Hemosiderin-laden macrophages in bronchoalveolar fluid [HPO term]
- Keratitis [HPO term]
- Localized pulmonary hemorrhage [HPO term]
- Middle age onset [HPO term]
- Multiple mononeuropathy [HPO term]
- Myalgia [HPO term]
- Oral ulcer [HPO term]
- Pediatric onset [HPO term]
- Perinuclear antineutrophil antibody positivity [HPO term]
- Pleuritis [HPO term]
- Polygenic inheritance [HPO term]
- Pulmonary infiltrates [HPO term]
- Respiratory insufficiency [HPO term]
- Retinal hemorrhage [HPO term]
- Rhinorrhea [HPO term]
- Sinusitis [HPO term]
- Skin ulcer [HPO term]
- Subglottic stenosis [HPO term]
- Tracheal stenosis [HPO term]
- Uveitis [HPO term]
- Weight loss [HPO term]
ORDO concept(s)
- Granulomatosis with polyangiitis [ORDO Disease]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Granulomatosis with Polyangiitis [MeSH Descriptor]
- Granulomatosis with Polyangiitis [NCIt concept]
- Granulomatosis with polyangiitis [MedDRA Preferred Term]
- Granulomatosis with polyangiitis [ORDO Disease]
- Granulomatosis with polyangiitis [Radlex concept]
- Granulomatosis with polyangiitis [ICD-11 Sub-sub category]
- Granulomatosis with polyangiitis (disorder) [SNOMED CT concept]
- Granulomatous polyangiitis [MedDRA LLT]
- WEGENER'S GRANULOMATOSIS [WHO-ART Preferred Term]
- Wegener granulomatosis [ACR pathology]
- Wegener granulomatosis [ACR pathology]
- Wegener granulomatosis [PASCAL descriptor]
- Wegener granulomatosis [Disease (Nov.)]
- Wegener's granulomatosis [ICD-9 code]
- Wegener's granulomatosis [ICD-10 Sub-category]
- Wegener's granulomatosis [MedDRA LLT]
- Wegeners granulomatosis [MedDRA LLT]
- granulomatosis with polyangiitis [MedlinePlus Topic]
- granulomatosis with polyangiitis [DO Concept]
- wegener's granulomatosis [SNOMED Notion]

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