Background/Purpose: Aims of therapy in incident AAV patients include ensuring rapid diagnosis, assessment of comorbidity, disease activity, and vasculitis damage before commencing treatment with a combination of high dose glucocorticoids (GC) with rituximab (RTX) or cyclophosphamide (CYC). It is believed to be important to achieve control of the vasculitis as soon as possible but also to avoid acute treatment-related morbidity as well as prevention of long term GC damage. This study aimed to examine clinical outcomes and adverse events in incident AAV patients in routine clinical practice in the EU.

Methods: This was a retrospective clinical review of 929 incident AAV (granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA)) patients from 4 European countries (399 physicians) who were diagnosed between 2014-17. Clinical data were reviewed at baseline, and at 1, 3, 6 and 12 months following commencement of induction therapy.

Results:

54% of patients had GPA, 46% MPA and mean age was 56.8 years (SD 14.2) with 53.7% male. Birmingham vasculitis activity score (BVAS) was used in only 12% of cases, but physicians reported 12% as mild/localized, 54% as moderate systemic and 34% as severe, life threatening. Comorbidities were common, with hypertension (44.9%), diabetes (18.1%), COPD/asthma (15.1%) and coronary arterial disease (10%) among the most frequently reported. Only 32.2% reported no comorbidities. Induction therapy varied with 59% receiving CYC, 24% RTX whilst 83% received GCs. As BVAS was not assessed in routine practice, clinical response was assessed as full (no vasculitis activity and GC taper on track), partial (reduction in vasculitis activity and major organ damage arrested) and no response (no improvement in vasculitis). Response rate varied and therapy-related adverse events were common.

Full response at 1 month was associated with good 12-month outcomes (81% full response) whereas a partial response at 1 month (56%) was associated with less favourable outcomes (58% full response at 12 months). Over the first 12 months of therapy, 6% of patients relapsed and required additional Treatment.

Conclusion:

Incident AAV patients frequently have comorbidity at diagnosis and vasculitis was rarely assessed using BVAS. Response to remission therapy was variable but early response is associated with a better response rate at 12 months. Therapy-related adverse events and infections are common, especially in the first 3 months. There is an unmet medical need for better response rates and reduction in toxicity of existing therapy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/variable-response-to-induction-therapy-and-significant-burden-of-treatment-adverse-events-over-the-first-12-months-in-incident-anca-associated-vasculitis-aav-patients-a-study-of-routine-cl/