Polyarteritis nodosa: an evolving primary systemic vasculitis

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ABSTRACT

Polyarteritis nodosa (PAN) is a primary form of vasculitis characterized by inflammation of primarily medium-sized arteries. Several key events have shaped the current spectrum of the disease including the separation of a subgroup with microscopic polyangiitis, the discovery of the association of hepatitis B, and the discovery of adenosine deaminase 2 deficiency (DADA2). With the discovery of secondary causes of PAN and changing nomenclature, the incidence of PAN has declined over time. Common manifestations include constitutional symptoms, skin involvement, peripheral neuropathy, gastrointestinal disease, and renal involvement. DADA2 is a genetic cause of medium vessel vasculitis that is important to distinguish from primary PAN as treatment with TNF inhibitors can prevent morbidity and mortality in those with a vasculitis phenotype. Treatment of systemic primary PAN involves the use of systemic immunosuppressive therapy largely guided by the severity of disease. With current treatment regimens, the prognosis has changed from a once uniformly fatal disease to a 5-year survival rate above 80%.

KEYWORDS:

• Polyarteritis nodosa
• medium vessel vasculitis
• adenosine deaminase 2 deficiency

Disclosure of any financial/other conflicts of interest

JMS has served as a consultant for ChemoCentryx, and the authors have no other financial or other conflicts of interest. Peer reviewers on this manuscript have no financial/other conflicts of interest to declare.

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Funding

No funding was received for the production of this manuscript.