Abstract: POS0832
Authors: Koutsianas C, et al.

zum Abstract

Management of ANCA vasculitides has significantly improved since the introduction of Rituximab in 2010. Thus, it would be interesting to learn, whether novel treatment strategies led to a reduction of mortality. This study addresses the overall long-term survival and all-cause mortality in a contemporary AAV patient cohort, the Greek ANCA registry.

Key content:
Data for 165 patients (989.38 patient-years) with a diagnosis of AAV (GPA n=95, 58%, MPA n=54, 33%, EGPA n=16, 9%) were analyzed (January 1, 1998 – January 10, 2022). 53% of patients were female, with a mean age of 65 (±16.4) years; the majority (97%) had generalized disease and were ANCA positive (76%). The mean follow-up since diagnosis was 5.9 (±5.1) years. At the end of follow-up, the overall mortality rate was 20% (33/165), whereas the cumulative mortality rates at 5 and 10 years were 24% and 26% respectively. Overall cumulative survival at 5 years was worse in patients with MPA (57%) compared to GPA (81%) and EGPA (92%), (pp<0.001).
Remarkably, there was no difference in long-term survival among those treated with different induction regimens including cyclophosphamide (CYC, n of deaths=24/83, 28.9%), rituximab (RTX, n=4/40, 10%) or the CYC+RTX combination (n=3/16, 18.7%). Furthermore, there was no difference in survival between relapsing (≥1 relapses) and non-relapsing (n=76) patients. Cumulative survival was worse in patients who initially presented with lung (66% vs. 90% at 5 years, p=0.007), kidney (56% vs. 96% at 5 years, p<0.001) and simultaneous lung and kidney (39% vs. 93% at 5 years, p<0.001) involvement. Among the 33 registered deaths, the most frequent causes were infections (52%), followed by cardiovascular events (24%), disease flares (14%) and malignancies (10%).

So far the induction and maintenance strategies using rituximab do not (yet?) translate into a better survival, nor did the authors find substantial changes in causes of death. The data demonstrate that ANCA vasculitides remain difficult to treat diseases and they highlight that combined lung and kidney involvement at baseline bears a particular risk.

Prof. Dr. Peter M. Villiger