Polyarteritis nodosa
ICD-10 M30.0; M30.2 · ICD-11 4A44.4

Treatment of Active Severe Polyarteritis Nodosa with Life- or Organ-Threatening Manifestations

Clinical Scenario

This protocol addresses newly diagnosed, active polyarteritis nodosa presenting with severe, life- or organ-threatening manifestations in a patient who is able to tolerate cyclophosphamide.

Defining Features
Life- or organ-threatening manifestations

Severe disease is defined by vasculitis involving critical organ systems — such as renal disease, mononeuritis multiplex, muscle disease, mesenteric ischemia, coronary involvement, or limb and digit ischemia. The clinical picture warrants prompt, aggressive remission induction.

Treatment Approach (partial overview)

Remission induction in this setting begins with high-dose intravenous pulse glucocorticoids, followed by oral glucocorticoids, combined with cyclophosphamide. Once remission is achieved, the protocol specifies transition to a less intensive immunosuppressive agent for a defined maintenance period — with planned discontinuation thereafter. Full sequencing, agent selection, and dosing details are in the structured protocol.

Treatment Goal

Clinical remission: complete absence of clinical signs or symptoms attributable to polyarteritis nodosa.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1002/art.41776

  1. Severe disease: Vasculitis with life- or organ-threatening manifestations (e.g., renal disease, mononeuritis multiplex, muscle disease, mesenteric ischemia, coronary involvement, limb/digit ischemia)
  2. For patients with newly diagnosed active, severe PAN, we conditionally recommend initiating treatment with intravenous (IV) pulse glucocorticoids over high-dose oral glucocorticoids.
  3. For patients with newly diagnosed active, severe PAN, we conditionally recommend initiating treatment with cyclophosphamide and high-dose glucocorticoids over high-dose glucocorticoids alone.
  4. Cyclophosphamide therapy should not continue indefinitely and should generally be limited to 3–6 months per course.
  5. For patients with newly diagnosed PAN who have achieved disease remission with cyclophosphamide, we conditionally recommend transitioning to another nonglucocorticoid immunosuppressive agent over continuing cyclophosphamide.
  6. For patients with PAN in remission who are receiving nonglucocorticoid immunosuppressive therapy, we conditionally recommend discontinuation of nonglucocorticoid immunosuppressive agents after 18 months over continued (indefinite) treatment.
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