| H01655 | |
| H number | H01655 |
| Name | Granulomatosis with polyangiitis; Wegener granulomatosis |
| Description | Granulomatosis with polyangitis (GPA) is an idiopathic, systemic inflammatory disease characterized by necrotizing granulomatous inflammation and pauci-immune small- vessel vasculitis of upper and lower respiratory tract and kidneys. The discovery of anti-neutrophil cytoplasmic antibodies (ANCAs) as a marker associated with GPA focused attention on the potential pathogenic role of these antibodies. Although it has been described that ANCAs recognise a variety of myeloid antigens, only antibodies that react with proteinase 3 (Pr3) and myeloperoxidase (MPO) have consistently been linked to vasculitis syndromes. Pr3-ANCA is the predominant autoantibody found in patients with GPA. The discovery of ANCA has made the diagnosis of this disease even more possible but there is almost always a need to confirm the diagnosis via histological examination of the lesional tissue. |
| Category | Immune system disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Rituximab [DR:D02994] |
| Comment | - |
| Other DBs | ICD-11: 4A44.A1 ICD-10: M31.3 MeSH: D014890 |
| Reference | PMID:23301777 AUTHORS Almouhawis HA, Leao JC, Fedele S, Porter SR TITLE Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment. JOURNAL J Oral Pathol Med 42:507-16 (2013) DOI:10.1111/jop.12030 PMID:15890083 AUTHORS Sarraf P, Sneller MC TITLE Pathogenesis of Wegener's granulomatosis: current concepts. JOURNAL Expert Rev Mol Med 7:1-19 (2005) DOI:10.1017/S146239940500921X |