This protocol addresses patients with active, severe polyarteritis nodosa who present with life- or organ-threatening manifestations and are unable to tolerate cyclophosphamide.
Severe manifestations in this setting include renal disease, mononeuritis multiplex, muscle disease, mesenteric ischemia, coronary involvement, and limb or digit ischemia.
Evidence-based guidance conditionally recommends initiating treatment with IV pulse glucocorticoids, combined with a nonglucocorticoid immunosuppressive agent, rather than glucocorticoids alone — the preferred alternative when cyclophosphamide is not an option.
DOI: 10.1002/art.41776
Severe disease: Vasculitis with life- or organ-threatening manifestations (e.g., renal disease, mononeuritis multiplex, muscle disease, mesenteric ischemia, coronary involvement, limb/digit ischemia)
For patients with newly diagnosed active, severe PAN who are unable to tolerate cyclophosphamide, we conditionally recommend treating with other nonglucocorticoid immunosuppressive agents and glucocorticoids over glucocorticoids alone.
For patients with newly diagnosed active, severe PAN, we conditionally recommend initiating treatment with intravenous (IV) pulse glucocorticoids over high-dose oral glucocorticoids.
In patients unable to tolerate cyclophosphamide, another agent, such as azathioprine or methotrexate, is recommended over glucocorticoid monotherapy.
Absence of clinical signs or symptoms attributed to PAN, on or off immunosuppressive therapy
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