This protocol addresses patients with active, severe polyarteritis nodosa presenting with life- or organ-threatening manifestations — including renal disease, mononeuritis multiplex, muscle disease, mesenteric ischemia, coronary involvement, or limb/digit ischemia — who could not tolerate cyclophosphamide and were therefore started on an alternative nonglucocorticoid immunosuppressive agent combined with glucocorticoids.
Initial treatment with IV pulse methylprednisolone followed by high-dose oral prednisone, plus azathioprine or methotrexate, has not achieved the required endpoint: clinical remission — absence of clinical signs or symptoms attributed to polyarteritis nodosa. This protocol defines the structured escalation step taken after that failure.
When azathioprine or methotrexate combined with glucocorticoids fails to induce remission in this setting, escalation to a more intensive nonglucocorticoid immunosuppressive agent alongside glucocorticoids is recommended — followed by a defined maintenance and transition strategy once remission is attained. The full sequence, agent selection, duration, and transition criteria are in the complete protocol.
Clinical remission: absence of clinical signs or symptoms attributed to polyarteritis nodosa, on or off immunosuppressive therapy.
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 severe PAN that is refractory to treatment with glucocorticoids and nonglucocorticoid immunosuppressive agents other than cyclophosphamide, we conditionally recommend switching the nonglucocorticoid immunosuppressive agent to cyclophosphamide, over increasing glucocorticoids alone.
cyclophosphamide therapy should not continue indefinitely and should generally be limited to 3–6 months per course.
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.
transitioning to another less toxic agent such as methotrexate or azathioprine is recommended once disease remission has been attained.
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