Abstract

Abstract Background AAV is a relapsing remitting condition and patients are at risk of organ damage from active AAV and therapy especially glucocorticoids (GC). The maintenance phase of AAV is critical for good long-term outcomes. This retrospective study examined maintenance definition, therapy and outcomes in patients in routine clinical practice. Methods 300 AAV patients managed by 100 UK physicians (40% rheumatologists) who completed induction therapy for organ or life threatening AAV and initiated maintenance between 2014-16 were studied. Data were collected at time maintenance was determined to begin by the physician and at 6, 12, 18 and 36 months. Results 56% had granulomatosis with polyangiitis; mean age 55.4 years 54% male. 61% had incident AAV and 39% relapsed. Physicians defined time of start of maintenance from treatment start with mean of 4.5 months, on basis of fixed time point 47%, starting new maintenance drug 27%, reaching full remission 16% and no specific criteria 9%. At this time 45% were in full remission vs 49% in partial and 6% refractory. Various maintenance regimes were used, 11% received rituximab (84% 6 monthly and 16% 12 monthly) at varying planned doses 61% 1g, 19% 500 mg and 16% 375 mg/m2, 4% not recorded. Remission rates varied and many patients experienced adverse events (AE) and infections, prolonged GC use was common. At most recent review patients had been followed for a mean of 49.2 months - 6% had died, 30% relapsed at least once, and 10% required renal replacement therapy. 62% had no vasculitis activity and were ANCA negative with 14% still experiencing active disease. 35% still received GCs - 19% > 5mg/ day. There was negative impact on functional status with 13% reducing working hours, 13% restricted social life, 4% leaving employment, 3% registered as disabled and 2% leaving full time education. Conclusion Maintenance therapy is variably defined at 4-5 months from start of remission induction therapy. Achieving full remission and preventing relapse are still problems and many patients require GC therapy to maintain remission. Infectious complications are a problem and there is significant negative impact on patient functional status. Disclosures D. Worthington Corporate appointments; Employee of Vifor Pharma. P. Rutherford Corporate appointments; Employee of Vifor Pharma. Shareholder/stock ownership; Vifor Pharma.

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