Treatment of adults with nonsevere eosinophilic granulomatosis with polyangiitis (EGPA)

ECG: electrocardiogram; EGPA: eosinophilic granulomatosis with polyangiitis; ENT: ear, nose, and throat; IL: interleukin.

* For patients with multiple-organ involvement or severe but not organ-threatening manifestations (eg, eosinophilic pancreatitis, painful eosinophilic enteritis without bleeding, or eosinophilic pulmonary infiltrates), higher doses of oral prednisone up to 1 mg/kg (typically up to 120 mg) daily or equivalent may be appropriate. For those receiving reinduction after a relapse, the dose is guided by their previous response and is typically the last dose that clearly achieved remission.

¶ Anti-IL-5/5R therapies used for the treatment of EGPA include mepolizumab 300 mg subcutaneously every four weeks or benralizumab 30 mg subcutaneously every four weeks. If unavailable, alternative options include methotrexate and mycophenolate mofetil.

Δ Lack of response to higher-dose glucocorticoids and anti-IL-5/5R therapy may indicate an alternative diagnosis. For those with definite EGPA in this situation, induction therapy with rituximab or cyclophosphamide is appropriate. Please see UpToDate content on treatment of EGPA for additional details.

◊ The pace of the taper requires adjustment based on individual patient response to therapy. Eosinophil counts and symptoms should be monitored carefully to avoid severe relapses. Patients who have had relapses may require a slower taper or a halt to the taper prior to completion. A table with an example glucocorticoid tapering regimen is available in UpToDate. Patients receiving a glucocorticoid dose equivalent ≥20 mg of prednisone per day may require pneumocystis pneumonia prophylaxis; refer to UpToDate topics on prevention of pneumocystis pneumonia.

§ In this context, remission means absence of clinical signs and symptoms attributable to active disease, with the exception of asthma and ENT symptoms, which must be greatly improved. Evaluation includes monitoring of eosinophilia and any organ system previously involved (eg, creatinine clearance and microscopic urinalysis; pulmonary function testing; chest imaging; ECG or echocardiogram; skin, eye, or ENT examination). Please see UpToDate content on treatment of EGPA for additional details.

¥ Alternative immunosuppressants other than IL-5 targeted therapies and glucocorticoids (eg, methotrexate or mycophenolate mofetil) should be held prior to induction therapy with cyclophosphamide or rituximab.

‡ Comparative efficacy of add-on therapies has not been well-studied, particularly in the context of background anti-IL-5/5R therapy. Rituximab is typically reserved for those with recurrent organ-threatening disease and/or recurrence despite a trial of another agent. Please see UpToDate content on treatment of EGPA for dosing information and additional less commonly used alternatives.

† Timing of trials off steroid-sparing agents is uncertain. Our authors typically stop any immunosuppressant agents (eg, methotrexate, mycophenolate, rituximab) before trying to stop anti-IL-5/5R therapy because the immunosuppressant therapies have greater adverse effects/toxicities. Anti-IL-5/5R therapies should be maintained for at least one year, and our authors favor longer maintenance for those with more severe initial disease or difficult-to-achieve remission. Patients who have had multiple relapses may require lifelong therapy. Please see UpToDate topics on EGPA treatment for additional detail regarding tapering steroid-sparing therapies.