Prognostic Factors for Survival and Relapse in ANCA-Associated Vasculitis with Renal Involvement: A Clinical Long-Term Follow-Up Study.

Agneta Ekstrand,Tuija Poussa,Tom Törnroth,Anna Salmela

doi:10.1155/2018/6369814

Abstract

Aim We describe the clinical pattern of ANCA-associated vasculitis (AAV) and assess long-term prognostic factors of patients and renal survival and relapse. Methods Data from 85 patients with renal biopsy-proven AAV at a single center with up to 20-year [median 16.2 years (95% CI 14.9-17.7)] follow-up were retrospectively collected. Results Overall, 55% of the patients had microscopic polyangiitis (MPA) and 45% had granulomatosis with polyangiitis (GPA). The histopathological classes were focal in 35%, crescentic in 26%, mixed in 20%, and sclerotic glomerulonephritis in 19% of the patients. As induction treatment, a combination of cyclophosphamide and corticosteroids was given to 82%, while a combination of azathioprine and corticosteroids was maintenance therapy in 79%. The twenty-year patient survival was 45%. In a multivariable analysis, age ≥58 years [hazard ratio (HR) 7.64, 95% CI 3.44-16.95] and myeloperoxidase (MPO) ANCA (HR 2.12, 95% CI 1.08-4.17) were associated with shorter patient survival time. Renal survival was 68% overall: 88% in focal, 71% in crescentic, 56% in mixed, and 37% in sclerotic class (p=0.01). Female sex (HR 0.26, 95% CI 0.10-0.73) was a predictor of improved renal survival, whereas GFR <30 ml/min and MPO-ANCA were associated with worse renal survival (HR 4.10, 95% CI 1.35-12.49 and HR 3.10, 95% CI 1.21-7.95, respectively). Relapse-free survival at 20 years was 10%. MPA was associated with a lower risk for relapse (HR 0.48, 95% CI 0.28–0.82). Conclusion We confirmed the improved patient and renal survival in AAV patients with glomerulonephritis, while relapse remained the primary challenge. Histopathological classification may be relevant for survival.

Highlights

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are the primary types of vasculitis that are associated with anti-neutrophil cytoplasm antibody (ANCA)
Three patients were lost to follow-up within 3 months of diagnosis, and they were excluded from the follow-up data analysis
In our cohort of renal associated vasculitis (AAV), a younger age was related to a favorable patient survival, and better glomerular filtration rate (GFR) at diagnosis was related to improved renal survival, as may be anticipated

Summary

Introduction

Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are the primary types of vasculitis that are associated with anti-neutrophil cytoplasm antibody (ANCA). Renal vasculitis is the most common severe manifestation of ANCA-associated vasculitis (AAV) typically presented with rapidly progressive glomerulonephritis (GN). Treatment per se may cause significant morbidity, and patients with impaired renal function may be prone to treatment-emergent adverse events [1]. Renal impairment at diagnosis predicts poor renal [2,3,4,5] and patient survival [6, 7]. One-half of the patients experience a relapse within five years after diagnosis [9, 10]

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