[Polyarteritis nodosa and Kawasaki syndrome : Vasculitis predominantly of medium size and small vessels]
Ulrike Sixdorf1, Elisabeth Märker-Hermann2
1Klinik Innere Medizin IV (Geriatrie und Rheumatologie), Helios Dr. Horst Schmidt Kliniken Wiesbaden GmbH, Ludwig Erhard-Str. 100, 65199, Wiesbaden, Deutschland. ulrike.sixdorf@helios-gesundheit.de.
Abstract:
Polyarteritis nodosa (PAN) and Kawasaki syndrome (KS) are rare forms of primary vasculitis with heterogeneous manifestations and courses of the disease. According to the Chapel Hill Consensus Conference 2012 they belong to the vasculitis of medium size vessels. In contrast to microscopic polyangiitis (MPA), PAN and KS do not affect microscopic vessels such as arterioles, venules or capillaries and are not associated with antineutrophil cytoplasmic antibodies (ANCA). The diagnostics are based on the typical constellation of clinical symptoms, on angiographic findings, the exclusion of other differential diagnoses and, in the case of PAN, in the histopathological confirmation. The therapeutic options of KS in childhood and PAN in adults and children, which are dependent on the severity and the prognosis, are presented.
Insights
Polyarteritis nodosa (PAN) and Kawasaki syndrome (KS) are rare medium-vessel vasculitides. Diagnostics involve clinical, angiographic, and histopathological findings, excluding microscopic polyangiitis (MPA) and ANCA.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Internal Medicine
Context:
- Polyarteritis nodosa (PAN) and Kawasaki syndrome (KS) are rare primary vasculitides.
- These conditions affect medium-sized vessels, as defined by the Chapel Hill Consensus Conference 2012.
- Unlike microscopic polyangiitis (MPA), PAN and KS do not involve microscopic vessels and are ANCA-negative.
Purpose:
- To differentiate PAN and KS from other vasculitides.
- To outline diagnostic criteria for PAN and KS.
- To present therapeutic strategies for PAN and KS based on disease severity.
Summary:
- PAN and KS are medium-vessel vasculitides distinct from MPA.
- Diagnosis relies on clinical presentation, angiography, and exclusion of other conditions; PAN requires histopathology.
- Treatment approaches for KS in children and PAN in all age groups are discussed.
Impact:
- Improved understanding of rare vasculitides.
- Guidance for clinicians in diagnosing and managing PAN and KS.
- Facilitates tailored treatment strategies for improved patient outcomes.
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