Abstract
Polyarteritis nodosa (PAN) is a primary systemic necrotizing vasculitis that preferentially involves medium-sized arteries. The etiology of PAN is unknown. Several viral infections, such as hepatitis B virus infection, may be associated with blood vessel inflammation, clinically and histologically indistinguishable from PAN. Clinical manifestations of PAN are heterogeneous and multisystemic. Peripheral nerve and skin are frequently involved. Other organs including gastrointestinal tract, kidney, heart, and central nervous system can be targeted, conveying a poorer prognosis. Laboratory markers reflecting a prominent acute-phase response are common but not specific. When histologic confirmation cannot be obtained, angiography of involved territories, preferentially abdominal, may disclose multiple aneurysm formation supporting the diagnosis of PAN. Current treatment policy includes high-dose corticosteroids, which are combined with immunosuppressive agents when critical organ involvement or life-threatening complications occur. IV pulse cyclophosphamide in the remission induction phase, later switched to a safer immunosuppressant for remission maintenance, is a frequently used therapeutic scheme. A recent consensus algorithm for the classification of PAN has attempted to address some of the shortenings of previous classification criteria and has also confirmed the low prevalence of PAN compared with other necrotizing systemic vasculitides.
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Hernández-Rodríguez, J., Cid, M.C. (2008). Polyarteritis Nodosa. In: Shoenfeld, Y., Cervera, R., Gershwin, M.E. (eds) Diagnostic Criteria in Autoimmune Diseases. Humana Press. https://doi.org/10.1007/978-1-60327-285-8_16
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DOI: https://doi.org/10.1007/978-1-60327-285-8_16
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