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Z Rheumatol. 2012 Nov;71(9):745-53. doi: 10.1007/s00393-012-0982-z.

[Update on granulomatosis with polyangitis (GPA, Wegener's granulomatosis)].

[Article in German]

Author information

1
UKSH, Campus Lübeck, Poliklinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Oskar-Alexander Str. 26, 24576, Bad Bramstedt, Deutschland. j.holle@klinikumbb.de

Abstract

Granulomatosis with polyangitis (GPA, Wegener's granulomatosis) is characterized by a granulomatous inflammation of the respiratory tract and a necrotizing ANCA-associated small to medium-size vessel vasculitis with a predilection for the lungs (pulmonary capillaritis) and kidneys (necrotizing glomerulonephritis). The disease evolves stage-wise and typically starts as inflammation of the respiratory tract followed by development of systemic vasculitis manifestations. Today, treatment is evidence-based and adapted according to activity and disease stage which has resulted in a significant improvement in long-term outcome. Early mortality during the first year of treatment poses one of the main problems and is a result of infections under immunosuppressive treatment. Furthermore, treatment of refractory disease activity which is often represented by granulomatous manifestations is still a challenge and may result in significant organ damage if not treated successfully.

PMID:
23138551
DOI:
10.1007/s00393-012-0982-z
[Indexed for MEDLINE]
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