Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis)

Abstract Granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis) is an autoimmune small vessel vasculitis which is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA). The hallmarks of this condition are systemic necrotising vasculitis, necrotising gr...

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Bibliographic Details
Published in:Journal of autoimmunity 2014-02, Vol.48, p.94-98
Main Authors: Lutalo, Pamela M.K, D'Cruz, David P
Format: Article
Language:English
Subjects:
Air Pollutants - adverse effects
Allergy and Immunology
ANCA
Autoimmune Diseases - classification
Autoimmune Diseases - diagnosis
Autoimmune Diseases - epidemiology
Biological and medical sciences
Fundamental and applied biological sciences. Psychology
Fundamental immunology
Granulomatosis
Granulomatosis with Polyangiitis - classification
Granulomatosis with Polyangiitis - diagnosis
Granulomatosis with Polyangiitis - epidemiology
Granulomatosis with Polyangiitis - genetics
Humans
Medical sciences
Myeloblastin - adverse effects
Polyangiitis
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Smoking - adverse effects
Staphylococcal Infections - classification
Staphylococcal Infections - diagnosis
Staphylococcal Infections - microbiology
Staphylococcus aureus - pathogenicity
Treatment
Vasculitis
Vasculitis - classification
Vasculitis - diagnosis
Vasculitis - enzymology
Vasculitis - epidemiology
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Summary:Abstract Granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis) is an autoimmune small vessel vasculitis which is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA). The hallmarks of this condition are systemic necrotising vasculitis, necrotising granulomatous inflammation, and necrotising glomerulonephritis. The aetiology of granulomatosis with polyangiitis is linked to environmental and infectious triggers inciting onset of disease in genetically predisposed individuals. Anti-neutrophil cytoplasmic antibodies are pathogenic and play an important role in the pathogenesis of this disease, although ANCA positivity is not essential for a clinical diagnosis of granulomatosis with polyangiitis. Granulomatosis with polyangiitis is diagnosed based on clinical manifestations of systemic vasculitis and histological evidence of necrotising vasculitis or granulomatous inflammation. This small vessel vasculitis may present as limited disease of the ears, nose and upper airways or mild, moderate or severe systemic disease. Immunosuppression and adjuvant therapies have contributed to the improved prognosis of granulomatosis with polyangiitis over the past decades. Treatment strategies are tailored to the severity of the disease. They are based on published evidence of the efficacy and safety of the immunosuppressive drugs indicated to manage active vasculitis and maintain clinical remission. This review will summarise the history, aetiology, pathogenesis, classification, diagnosis and management of granulomatosis with polyangiitis.
ISSN:0896-8411
1095-9157
DOI:10.1016/j.jaut.2014.01.028