Background/Purpose:

Eosinophilic granulomatosis with polyangiitis (EGPA) often presents with persistently active disease requiring chronic glucocorticoid therapy or it may have a relapsing course with partial or no response to traditional immunosuppressive agents.  Severe disease frequently responds to cyclophosphamide but its use is limited by the significant toxicity associated with prolonged therapy.  The maintenance of disease remission presents a challenge, as patients often require unacceptable doses of glucocorticoids to control asthma or active disease.  Rituximab (RTX) is an effective agent used for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).  Recent retrospective reports have suggested that RTX may also be effective for EGPA.  The role of RTX for EGPA is still unclear, including whether repeated courses of RTX may benefit individuals with relapsing disease.

Methods:

We present our experience using RTX for EGPA and present the available worldwide data on RTX for EGPA.

Results:

We identified 56 patients meeting ACR or Revised International Consensus Criteria for EGPA at our institution, 6 of whom received RTX.  Five of 6 patients (83%) treated with RTX significantly improved.  The remaining patient experienced respiratory distress during the first infusion and did not complete the infusion.  These 5 patients had long follow-up (mean 53 months) and received multiple courses of RTX (mean 4.6 courses) for either disease relapse or remission maintenance.  We identified 65 cases of EGPA treated with RTX published in the literature with 83-95% response rate using variable outcome measures.  These patients received fewer courses of RTX (mean 1.6) and had shorter follow-up (mean 15 months).  There were 3 infusion reactions (5%) and 18 infections (28%), 8 of which were severe (12%).  Patients from our cohort and from the literature were able to reduce prednisone dose from a mean baseline of 28mg/day to 6.6mg/day and from 18.9mg/day to 8.1mg/day, respectively.

Discussion:

This series provides data on the use of multiple courses of RTX for EGPA.  It is also the first series to provide follow-up beyond 3 years for RTX use in EGPA.  It is noteworthy that further reductions in prednisone were observed in patients who received repeated courses of RTX as ongoing therapy.

Conclusion:

Although the role of RTX for EGPA is still unclear, a small but growing number of reports suggest that RTX may be an effective treatment option for EGPA that is severe, relapsing or refractory to other medications.  Long-term treatment of EGPA using multiple courses of RTX may allow for further reductions in prednisone and thereby reduce associated morbidity.  A multicenter prospective trial should be considered to better define both the short-term and long-term efficacy and safety of RTX in patients with EGPA.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-use-of-rituximab-for-eosinophilic-granulomatosis-with-polyangiitis/