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Abstract: PO1609

Long-Term Outcome in Patients with ANCA-Associated Vasculitis (AAV): The Monocentric Experience of Brescia

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Salviani, Chiara, Nephrology Unit, ASST Spedali Civili di Brescia, Brescia, Italy
  • Gregorini, Gina Alessandra, Nephrology Unit, ASST Spedali Civili di Brescia, Brescia, Italy
  • Tedoldi, Gianluca, Universita degli Studi di Brescia, Brescia, Lombardia, Italy
  • Alberici, Federico, Nephrology Unit, ASST Spedali Civili di Brescia, Brescia, Italy
  • Scolari, Francesco, Nephrology Unit, ASST Spedali Civili di Brescia, Brescia, Italy
Background

Renal involvement in AAV is common, ranging between 80 and 90%; of note, up to 40% of patients (pts) with renal involvement will develop End-Stage Renal Disease (ESRD).
We explored prognostic factors of renal and overall long-term survival in AAV pts with renal involvement.

Methods

Monocentric, retrospective study, including all pts with clinical or histological diagnosis of AAV with renal involvement followed at our Unit from 1990 to 2019, with follow-up (f-u) ≥12 months.

Results

We identified 281 patients. Median f-u was 75 months (IQR 33-141). Most pts were classified as MPA (71%), followed by GPA (26%) and eosinophilic granulomatosis with polyangiitis (3%). ANCA were positive in 97% (anti-MPO in 66%, anti-PR3 in 31%).
At onset, median creatinine was 3.5 mg/dl (IQR 1.9-6.7) and proteinuria 1.1 g/24h (IQR 0.5-2); 20% of pts required haemodialysis (HD), with subsequent recovery of renal function in 55% of them.
Induction therapy consisted of oral corticosteroids for all pts, with iv pulses >1g in 38% of cases; plasma-exchange in 27%; cyclophosphamide in 57% and rituximab in 33% of pts.
Relapses were experienced in 31% of pts, with renal flares in 20%.
Renal survival was 87%, 79% and 73% at 1,5 and 10 years, respectively. At multivariate Cox regression analysis, clinical diagnosis of MPA (HR 5.116, CI 3.437-7.408, p<0.0001) and HD requirement at onset (HR 13.469, CI 9.050-19.504, p<0.0001) were predictors of ESRD.
Overall survival was 90%, 77% and 56% at 1, 5 and 10 years, respectively. Most deaths were due to infections (33%), followed by cardiovascular (CV) diseases (20%) and malignancies (13%).
At multivariate Cox regression analysis, age (HR 5.724, CI 3.846-8.288, p<0.0001) and HD requirement at onset (HR 2.902, CI 1.949-4.202, p<0.0001) were predictors of mortality.

Conclusion

Despite several therapeutic advances, AAV with renal involvement are still characterised by poor prognosis, especially in pts requiring HD at onset and of older age; infection, CV diseases and malignancies were the main causes of death.