Polyarteritis Nodosa: State of the art

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that preferentially affects medium-sized vessels. The idiopathic form has become rare. Its treatment relies on corticosteroid therapy and is combined with cyclophosphamide infusions for severe forms. Secondary PANs were mainly associated with hepatitis B virus infection; they were treated with plasma exchange and antivirals in combination with short-term corticosteroid therapy. Other secondary forms of PAN are now becoming more common, such as those due to blood disorders. More recently, a monogenic form linked to adenosine deaminase-2 mutations has been identified. It requires treatment with TNF inhibitors to decrease the occurrence of ischemic central nervous system complications, which make it serious. Once remission is obtained, relapses are typically rare during PAN and affect 28% of idiopathic PANs, within an average of 26 months from the diagnosis. The prognosis has improved considerably, with 5- and 10-year survival rates of 83% and 74%.