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Clinical manifestations and diagnosis of polyarteritis nodosa in adults

Author
Peter A Merkel, MD, MPH
Section Editor
Gene G Hunder, MD
Deputy Editor
Monica Ramirez Curtis, MD, MPH

INTRODUCTION

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries [1,2]. Unlike some other vasculitides (eg, microscopic polyarteritis, granulomatosis with polyangiitis [Wegener’s]), polyarteritis nodosa is not associated with antineutrophil cytoplasmic antibodies (ANCA) [3]. (See "Overview of and approach to the vasculitides in adults".)

Patients typically present with systemic symptoms. The kidneys, skin, joints, muscles, nerves, and gastrointestinal tract are commonly involved, usually in some combination and sometimes all at once by the time of diagnosis. PAN can affect virtually any organ but has a striking tendency to spare the lungs. Clinical variants or subsets of PAN include single-organ disease and cutaneous-only PAN.

The spectrum of disease known as PAN has evolved and narrowed substantially due to the identification and classification of other forms of vasculitis that had previously been considered PAN. Most importantly, the establishment and acceptance of microscopic polyangiitis as a distinct disease, and the routine availability and recognition of ANCA testing as critical to diagnosing vasculitides other than PAN, have led to more patients with vasculitis being classified as not having PAN. Patient cohorts of PAN included in case series and other research studies published prior to the 1990s almost certainly included a mixture of the newer definition of PAN with microscopic polyangiitis and possibly other forms of vasculitis. Thus, case series of PAN published after this time period that specifically exclude patients with MPA provide important updated information about this disease [4]. (See "Overview of and approach to the vasculitides in adults" and "Clinical spectrum of antineutrophil cytoplasmic antibodies".)

The clinical manifestations and diagnosis of PAN will be reviewed here. Treatment is presented separately. (See "Treatment and prognosis of polyarteritis nodosa".)

Microscopic polyangiitis and other ANCA-associated systemic vasculitides (eg, granulomatosis with polyangiitis [Wegener’s] and eosinophilic granulomatosis with polyangiitis [Churg-Strauss]) that characteristically affect small vessels such as arterioles, capillaries, and venules, as well as muscular arteries, are discussed separately. (See "Overview of and approach to the vasculitides in adults" and "Pathogenesis of granulomatosis with polyangiitis and related vasculitides" and "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis" and "Clinical spectrum of antineutrophil cytoplasmic antibodies".)

                      

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Literature review current through: Nov 2016. | This topic last updated: Wed Dec 16 00:00:00 GMT 2015.
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