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Published on: August 21, 2017
[ANCA-associated vasculitis]
1Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Oskar-Alexander Str. 26, 24576, Bad Bramstedt, Deutschland, j.holle@klinikumbb.de.
Insights
ANCA-associated vasculitides (AAV) involve small-vessel inflammation and may be diagnosed without ANCA antibodies. Treatment focuses on remission induction and maintenance using conventional immunosuppressants and newer options like rituximab.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Context:
- Vasculitides are systemic rheumatic diseases affecting various vessel sizes.
- ANCA-associated vasculitides (AAV) encompass granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis.
- These conditions manifest in small- to medium-sized vessels across organ systems.
Purpose:
- To outline the classification and diagnostic considerations for vasculitides.
- To describe the treatment principles for ANCA-associated vasculitides.
- To highlight the evolving therapeutic landscape, including novel agents.
Summary:
- AAV are characterized by inflammation of small- to medium-sized vessels and may present with or without detectable anti-neutrophil cytoplasmic antibodies (ANCA).
- Treatment strategies are guided by disease activity and stage, involving remission induction followed by maintenance therapy.
- Established treatments include glucocorticoids and conventional immunosuppressants, with rituximab recognized as a significant advancement since 2013.
Impact:
- Improved understanding of AAV classification and diagnostic nuances.
- Standardized treatment approaches based on clinical trial evidence.
- Integration of targeted therapies like rituximab into AAV management protocols.
Abstract:
The vasculitides represent one group of the systemic rheumatic diseases. Among the vasculitides we distinguish between large- (i.e. giant cell arteritis), medium- (i.e. polyarteritis nodosa) and small-vessel vasculitides (i.e. ANCA-associated vasculitides). Granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis belong to the ANCA-associated vasculitides. They share the features of vasculitic manifestations in small- to medium-sized vessel beds (which can occur in almost any organ system) and the presence of ANCA, the detection of which, however, is not necessarily mandatory. The treatment of AAV depends on disease stage and activity and is carried out on the basis of randomized controlled trials with an initial regimen aimed at inducing remission followed by maintenance treatment. In addition to glucocorticoids, conventional immunosuppressants (such as methotrexate, azathioprine and cyclophosphamide) form the basis of treatment, whereby rituximab, first licensed for the treatment of severe active GPA and MPA in 2013, has emerged as new treatment option.
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