P2_1 Management and outcomes of ANCA-associated vasculitis in unselected new cases within a large health region of England

Fiona Pearce, Catherine Mary McGrath, Ravinder Sandhu, Jon Packham, Karen Obrenovic, Richard Watts, Peter Lanyon

Research output: Contribution to journalAbstractpeer-review


Background: Most ANCA-associated vasculitis (AAV) outcome data derives from highly-selected tertiary centres or clinical trials. These results may not be generalizable to routine practice.

Methods: Rheumatology and Renal units within Midlands and East of England were invited to undertake retrospective case-note audit of patients newly-diagnosed with AAV between April 2013-December 2014. Data were collected on time to diagnosis and treatment, systems involved, remission-induction, and outcomes. Odds ratio (OR) for infection estimated using logistic regression, and hazard ratio (HR) for death estimated using cox regression were both adjusted for confounders (age, renal involvement).

Results: Cases were contributed by 20 (59%) of 34 units (Table 1). A cohort of 130 newly- diagnosed adult patients were identified in an adult catchment population of 6,980,000. This equates to incidence rate of 10.6 per million person years, indicating identification of approx. 50% of all incident cases. 59% came from Rheumatology and 41% from Renal units. 72 (55%) were diagnosed as inpatients, and 58 (45%) as outpatients. Delay from first symptoms to diagnosis was median 2.6 (interquartile range 1.2-6.1) months. It was shorter in sicker patients (defined as inpatients, 1.8 (0.9-3.7) compared to outpatients, 4.1 (2.0-12.6). Among inpatients, delay from admission to diagnosis ranged from 0-53 days (median 6, IQR 3-10.5). 1 year survival was 90.8%. Cyclophosphamide (CYC) was used in 99 (76%), Rituximab in 6 (5%), and other agents in 25 (19%). Of 99 newly-diagnosed CYC-treated patients, 74 (75%) received IV and 25 (25%) oral. 24 (24%) had infections requiring hospitalisation during or within 6 months of CYC. 1 year survival in this subgroup was 87.9%. Compared to IV, the crude OR for infection with oral CYC was 2.2 (0.8-6.0) and HR for death was 2.3 (0.8-6.6). Once adjusted for age and renal involvement, OR for infection remained elevated at 1.8 (0.6-5.1) and HR for death was 1.7 (0.5-5.3).

Conclusions: A need to improve care is highlighted by significant mortality and infection. Although limited by lack of power, and at risk of confounding by indication, it raises the hypothesis that in routine practice oral CYC may be associated with increased risk of death and infection requiring hospitalization.
Original languageEnglish
Pages (from-to)iii104-iii109
Issue numbersuppl 3
Early online date10 Mar 2017
Publication statusPublished - 27 Mar 2017
Event18th International Vasculitis & ANCA Workshop - Tokyo, Japan
Duration: 25 Mar 201728 Mar 2017


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