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Treatment and prognosis of polyarteritis nodosa

INTRODUCTION

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects medium-sized muscular arteries and often involves small muscular arteries [1,2]. The approach to treatment of PAN depends upon the following variables, which require assessment before beginning therapy:

The level of disease severity

The presence of isolated cutaneous PAN or other isolated/single-organ disease

The presence or absence of viral hepatitis

The optimal therapy of PAN remains uncertain, and studies of treatment for PAN have been complicated by the admixture of patients with PAN, microscopic polyangiitis (MPA), and sometimes eosinophilic granulomatosis with polyangiitis (Churg-Strauss) within study cohorts [3-5]. The efficacy of glucocorticoids in the majority of patients with mild disease, and of glucocorticoids plus cyclophosphamide in patients with more severe disease, has been well-demonstrated in observational studies in PAN, but there are few randomized trials [1,3,4,6-9]. The treatment approach in PAN is also derived from indirect evidence of the efficacy of various medications and drug regiments in other forms of necrotizing vasculitis, particularly MPA and granulomatosis with polyangiitis (Wegener’s). (See "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Maintenance immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis".)

                             

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Literature review current through: Mar 2014. | This topic last updated: Mar 27, 2014.
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