Background/Purpose
Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and Churg-Strauss Syndrome (CSS) are syndromes known as ANCA-associated vasculitis (AAV). In addition to immunosuppressive therapy, plasma exchange (PLEX) may be indicated in patients with pulmonary hemorrhage and/or severe renal insufficiency. However, PLEX may be associated with serious adverse effects such as infection. The objective of this study was to characterize and examine outcomes in patients with AAV treated with PLEX, in addition to corticosteroid and cytotoxic agents, at a major referral centre for PLEX.
Methods
A retrospective chart review was performed on all patients with AAV treated with PLEX at a major referral centre for PLEX between January 1, 2002 to May 31, 2012. Patients with GPA, MPA, CSS, systemic p-ANCA vasculitis, and systemic c-ANCA vasculitis were included while those with incomplete 3 and 12 month follow-up were excluded. Demographic, clinical, laboratory, and radiographic data from electronic and paper medical records were collected. Acute kidney injury (AKI) was defined as an increase in serum creatinine by ≥26.5 umol/L within 48 hours, or a ≥1.5 times increase above baseline serum creatinine within 7 days. Disease activity was assessed by the Birmingham Vasculitis Activity Score (BVAS) (v.3). Primary outcomes were survival at 1 year, dialysis dependence at 1 year, and dialysis dependence at 3 months from initiation of PLEX. The study was approved by the institution’s Research Ethics Board.
Results
Forty-nine patients with AAV were treated with PLEX during the study period. Outcomes are reported for 43 patients, which excludes 4 patients lost to follow up and 2 patients with 3 and 12 month follow up that occurred outside the study period. 58% were male, and the median age was 59 years (range 25-83). This was the first presentation of AAV for 60%. GPA, MPA, CSS, systemic p-ANCA vasculitis, and systemic c-ANCA vasculitis was the primary diagnosis in 39%, 28%, 0%, 5%, and 28% of patients. Both pulmonary hemorrhage and AKI were present in 66%. The mean BVAS (v.3) score at presentation was 17.9. Triple therapy with systemic corticosteroid, cyclophosphamide, and PLEX occurred in 90%. Survival at 1 year, dialysis dependence at 1 year, and dialysis dependence at 3 months was 88%, 28%, and 37%, respectively. Renal recovery amongst those who were dialysis-dependent at presentation was 37% and 47% at 3 months and 1 year, respectively. Infection, bleeding (non-pulmonary hemorrhage), symptomatic hypotension, and catheter-related thrombosis occurred in 44%, 20%, 5%, and 2% of patients.
Conclusion
With triple therapy, 88% of patients with AAV survived at least 1 year. Almost 50% of patients who were dialysis-dependent on presentation experienced renal recovery after 1 year. An international randomized controlled trial is currently underway to investigate the specific role of PLEX in the treatment of AAV.
Disclosure:
J. Ueng,
None;
K. Pavenski,
None;
L. Rubin,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/outcomes-of-triple-therapy-plasma-exchange-cyclophosphamide-and-systemic-corticosteroid-for-anti-neutrophil-cytoplasm-antibody-anca-associated-vasculitis/