Polyarteritis Nodosa Guidelines

Updated: Oct 07, 2022
  • Author: Dana Jacobs-Kosmin, MD, FACP; Chief Editor: Herbert S Diamond, MD  more...
  • Print
Guidelines

Guidelines Summary

The American College of Rheumatology (ACR) and the Vasculitis Foundation (VF) have released joint guidelines on the management of polyarteritis nodosa (PAN). [81]

Imaging

The guidelines offer the following conditional recommendations [81] :

  • Abdominal vascular imaging can aid in establishing a diagnosis and determining the extent of disease
  • Follow-up abdominal vascular imaging for patients with a history of severe abdominal PAN who become asymptomatic
  • A deep-skin biopsy is preferred over a superficial skin punch biopsy to aid in establishing a diagnosis of PAN involving the skin
  • A combined nerve and muscle biopsy is preferred over a nerve biopsy alone to aid in establishing a diagnosis of peripheral neuropathy and suspected PAN
  • Serial neurologic examinations instead of repeated electromyography/nerve conduction studies (eg, every 6 months) should be used to monitor disease activity in patients with a history of peripheral motor neuropathy secondary to PAN

Treatment

Conditional recommendations for patients with newly diagnosed severe  active disease include [81] :

  • Initiating treatment with IV pulse glucocorticoids (GCs) over high-dose oral GCs
  • Initiating treatment with cyclophosphamide and high-dose GCs over high-dose GCs alone
  • Initiating treatment with cyclophosphamide and GCs over rituximab and GCs
  • Treat with other non-GC immunosuppressive agents and GCs over GCs alone for patients unable to tolerate cyclophosphamide
  • The use of plasmapheresis combined with cyclophosphamide and GCs is NOT recommended 

For patients with newly diagnosed nonsevere active disease, the guidelines conditionally recommend treatment with non-GC immunosuppressive agents and GCs over GCs alone. For patients with PAN in remission who are receiving non-GC immunosuppressive therapy, discontinuation of non-GC immunosuppressive agents after 18 months over continued (indefinite) treatment is conditionally recommended. [81]

Physical therapy is conditionally recommended for patients with nerve and/or muscle involvement.

The guidelines strongly recommend treatment with tumor necrosis inhibitors over GCs alone for patients with DADA2.