What are the approach considerations in the treatment of polyarteritis nodosa (PAN)?

Updated: Dec 17, 2020
  • Author: Dana Jacobs-Kosmin, MD, FACP; Chief Editor: Herbert S Diamond, MD  more...
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The treatment of polyarteritis nodosa (PAN) has improved dramatically. Previously, untreated PAN was usually fatal within weeks to months, with mortality often associated with kidney failure, cardiac complications, or gastrointestinal (GI) complications. Therefore, early diagnosis and treatment are critical in PAN.

Currently, corticosteroids are the cornerstone of treatment. The addition of cyclophosphamide is the standard of care for patients with idiopathic PAN whose disease is steroid refractory or includes major organ involvement. This combination can provide prolonged survival for these patients.

Cyclophosphamide is not routinely recommended in hepatitis B–related PAN, as the use of steroids with cyclophosphamide in these patients has been demonstrated to enhance viral replication. Instead, treatment for hepatitis B–related PAN consists of schemes that include corticosteroids with antiviral agents and plasmapheresis. Antiviral drugs used include vidarabine or interferon alpha-2b.

Biologic agents have been investigated in patients with steroid-refractory and recurrent PAN. Case reports have described response to treatment with tumor necrosis factor inhibitors, [17] including infliximab [60, 61] and etanercept. [62]  The interleukin-6 antagonist tocilizumab has been used successfully in refractory cases. [63, 64, 65] Single case reports describe successful use of the anti-CD20 agent rituximab [66] and the Janus kinase (JAK) inhibitor tofacitinib in refractory PAN. [66] Plasma exchange has been used in a few patients with severe PAN. [67]

Surgical care

Surgery may be necessary for GI manifestations of PAN, including bowel ischemia, cholecystitis, and appendicitis. Microcoil embolization of cerebral aneurysm may be indicated. Postsurgical care may be needed for patients with PAN who develop bowel infarction.

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