Polyarteritis Nodosa: State of the art - 29/06/22
Highlights |
• | Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly affecting medium-sized vessels. |
• | Its treatment relies on corticosteroid therapy, combined with cyclophosphamide infusions for severe forms. |
• | Secondary forms of PAN have become more common, including those associated with the hepatitis B virus and other infections and blood disorders. |
• | A monogenic form linked to adenosine deaminase-2 mutations was recently described. This form requires treatment with TNF inhibitors to reduce the ischemic central nervous system complications that make it serious. |
Abstract |
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%.
El texto completo de este artículo está disponible en PDF.Keywords : Polyarteritis nodosa, Necrotizing vasculitis, Hepatitis B infection, Adenosine deaminase-2 mutations, Diagnosis, Treatment
Esquema
Vol 89 - N° 4
Artículo 105320- juillet 2022 Regresar al númeroBienvenido a EM-consulte, la referencia de los profesionales de la salud.
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