Background/Purpose: After successful remission induction AAV is a relapsing remitting long term condition and patients are at risk of organ damage from both active AAV and therapy in particular from glucocorticoids (GC). The remission maintenance phase of AAV is critical for good long term outcomes. This retrospective study aimed to examine the definition of maintenance, therapies used and  outcomes in AAV patients managed in routine clinical practice

Methods: AAV patients from 4 European countries (310 physicians) who completed induction therapy for organ or life threatening AAV and initiated maintenance therapy between 2014-16 were studied. Data were collected at the time maintenance was determined to begin by the physician and then at following 6, 12, 18 and 36 months

Results: 929 patients were studied  – 51% of patients had granulomatosis with polyangiitis,; mean age 54 years with 54% male. 49% were studied from incident AAV and 51% from a relapse. Physicians defined the start of maintenance with mean of 5.6 months from induction start on basis of fixed time point 38%, starting of new drug for maintenance 27%, reaching full remission 26% and no specific criteria 9%. At this time 45% were in full AAV remission vs 49% in partial and 6% refractory. Over 36 months from when maintenance was defined, 84% were in remission but 10% had major relapse requiring re-induction therapy and left follow up, 6% died (in 2/3 of cases at time of relapse).  There is variation in the drugs used for maintenance therapy in real world practice. At 36 months, 9% of AAV patients were receiving renal replacement therapy and CKD was  reported in 17% of patients vs 7% at start of remission induction therapy and osteoporosis also increased 15% vs 7%.  55% of patients had no AAV activity, 28% positive ANCA serology but no clinical disease, 8% local AAV, 6% mild to moderate systemic disease and 3% moderate to severe systemic disease. Active AAV was see most commonly in kidney (29%), lung (13%) and sinuses (8%). There was negative impact on patient functional status after 36 months with 13% having reduced working hours, 13% restricted social life, 6% had to leave employment, 5% were registered as disabled and 2% had to leave full time education

Conclusion: Maintenance therapy in AAV has variable definitions but typically begins after 6 months of remission induction therapy. Relapse of varying degree is still a clinical problem and many patients require ongoing GC therapy to maintain remission. Infectious complications are a problem and there is significant negative impact on patient functional status over time. There is an ongoing need for new targeted therapies in AAV to improve clinical and patient functional status

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/maintenance-treatment-in-anca-associated-vasculitis-in-real-world-clinical-practice-burden-of-disease-use-of-glucocorticoids-and-impact-on-patient-functional-status-remain-major-problems/