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Abstract: FR-PO648

Do the Benefits of Subcutaneous Immunoglobulin Therapy for Secondary Hypogammaglobulinemia in ANCA Vasculitis Extend Beyond Infection Prevention?

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Churilla, Bryce Matthew, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Aqeel, Faten Faisal, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
  • Geetha, Duvuru, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
Background

Rituximab (RTX) is an effective treatment for induction and maintenance of remission in ANCA-associated vasculitides (AAV). However, hypogammaglobinemia and increased infection risk remain a concern. Subcutaneous immunoglobin (SCIG) therapy has been shown to improve this RTX induced side effect, but its impact on disease remission is not known.

Methods

This observational report highlights a single-center series of 5 AAV patients treated with RTX for remission induction. These individuals received SCIG for moderate to severe IgG deficiency. We examined the effects of SCIG on serum IgG level, disease remission, rituximab use, relapses and infections in this cohort.

Results

The median age was 68 yrs, 3 had relapsing disease and 3 were PR3 ANCA positive. At baseline, 4 patients had experienced recurrent infections. Serum IgG levels improved with SCIG administration in all patients and recurrence of infection markedly improved (Table 1). Post SCIG, RTX was either no longer needed or ultimately discontinued. All patients remained in disease remission with SCIG administration with no relapses noted.

Conclusion

SCIG therapy has been shown to improve hypogammaglobinemia in RTX treated AAV. However, our report highlights a potential benefit in minimizing relapses in these patients. This finding which is of significance in management of AAV needs to be confirmed in clinical trials.

IDAge Year, SexMaintenance TreatmentNumber of InfectionsNumber of AAV RelapsesCurrent Immunosuppression
  Before SCIGAfter SCIGBefore SCIGAfter SCIGBefore SCIGAfter SCIG 
168, FRTX every 6 months and then every 4 monthsRTX stopped512NonePrednisone
279, FNoneNone4NoneNoneNoneNone
369, FAZA, PrednisoneNone5NoneNoneNonePrednisone
416, MAZA, LEF, Prednisone, RTX 1000 mg every 6 monthsRTX dose and duration decreased initially and then stoppedNoneNone6NoneNone
550, MRTX every 6 monthsRTX duration decreased initially and then stopped3None1NoneNone

ANCA: anti-neutrophil cytoplasmic antibody, AAV: ANCA-associated vasculitis, SCIG: subcutaneous immunoglobulins, F: female, M: male, RTX: Rituximab, AZA: Azathioprine, LEF: Leflunomide