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[Vasculitis 1998: new aspects in diagnosis and therapy]
1Sektion Nephrologie der Medizinische Klinik, Universität Heidelberg, Deutschland.
Insights
Systemic vasculitis classification was updated. Serum procalcitonin aids in distinguishing disease activity from infection, while ANCA testing offers reliable diagnosis comparable to histology for vasculitis.
Area of Science:
- Rheumatology and Immunology
- Nephrology
Background:
- The Chapel Hill conference updated systemic vasculitis classification.
- Standardized scoring systems (BVAS, VDI) for disease activity and organ damage are crucial.
- Differentiating bacterial infection from active vasculitis is clinically important.
Purpose of the Study:
- To review current understanding and diagnostic approaches for systemic vasculitides.
- To highlight the role of ANCA testing and serum procalcitonin in diagnosis and management.
- To discuss the pathogenesis and therapeutic strategies for ANCA-associated vasculitides.
Main Methods:
- Review of recent studies and clinical observations.
- Analysis of diagnostic markers including ANCA (C-ANCA/PR3-ANCA, P-ANCA/MPO-ANCA) and serum procalcitonin.
- Discussion of pathogenetic mechanisms involving neutrophils and T-cells.
- Overview of ongoing European therapeutic trials (EUVAS).
Main Results:
- Serum procalcitonin effectively differentiates active vasculitis from infection.
- ANCA testing demonstrates diagnostic reliability comparable to histology for vasculitis.
- ANCA positivity can occur in non-vasculitic conditions, requiring careful diagnosis.
- Association between ANCA and IgA nephropathy (IgA-GN) is noted, with differing clinical courses.
Conclusions:
- ANCA-positive vasculitides are likely T-cell mediated, with neutrophils expressing class II antigens and responding to infectious stimuli.
- Therapeutic strategies are evolving to optimize treatment, particularly limiting cyclophosphamide use to active disease stages.
Abstract:
The Chapel Hill conference established the most actual classification of systemic vasculitides. Disease activity and organ damage were standardized by means of a new scoring system (BVAS; VDI). Bacterial infection is frequent during the active phase of vasculitis. The measurement of serum procalcitonin helps to differentiate between disease activity and infection. In a recent study the European vasculitis group (EC-BCR project) found that the presence of C-ANCA/PR 3-ANCA or P-ANCA/MPO-ANCA is as reliable as histological proof of vasculitis. In rare cases ANCA is positive in diseases other than vasculitis, which has to be kept in mind for diagnosis and therapy. The association between ANCA and IgA nephropathy is exciting. The occurrence of IgA-GN in patients with Wegener's granulomatosis is usually a secondary event whereas in patients with rapidly progressive IgA-GN microscopic polyangiitis follows. Many observations support the view that ANCA-positive vasculitides are T-cell mediated diseases. Our investigations demonstrate that neutrophils express class II antigens during active disease and are able to trigger T-cell activation via infectious stimuli. With regard to therapy of ANCA-positive vasculitides several strategies are currently being examined in different European countries (EUVAS) with the aim to limit cyclophosphamide to the active stage of the disease.