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Abstract Number: 2381

ANCA-associated Vasculitis and Renal Disease in a Multidisciplinary Outpatient Clinic in Northern Spain

Salma Al Fazazi1, Fabricio Benavides2, Vanesa Calvo Río3, maria rodriguez Vidriales2, clara escagedo Cagigas2, monica renuncio garcia2, luis martin penagos4 and Ricardo Blanco5, 1Hospital Universitario Puerta Del Mar, Cádiz, Spain, 2Hospital Universitario Marqués de Valdecilla, Santander, Spain, 3Valdecilla Hospital, Santander, Spain, 4Division of Nephrology, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-DIVAL, Santander, Spain, 5Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

Meeting: ACR Convergence 2023

Keywords: ANCA, ANCA associated vasculitis, Nephritis, Vasculitis

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Session Information

Date: Tuesday, November 14, 2023

Title: (2370–2386) Vasculitis – ANCA-Associated Poster III: Biomarkers & Renal Outcomes

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: The severity of clinical features and outcomes in previous series of patients reported with renal disease related to ANCA-associated vasculitis (AAV) vary greatly, probably due to selection bias.

To establish the actual clinical spectrum of renal disease due to AAV in a multidisciplinary outpatient clinic in Northern Spain.

Methods: Review of 132 patients classified as having AAV between 1994 to 2022; Granulomatosis with polyangiitis (GPA), Microscopic polyangiitis (MPA) or Eosinophilic Granulomatosis polyangiitis (EGPA) was classified according to 2022 ACR/EULAR classification criteria.

Results: Renal disease was observed in 82 of 132 (62.1%) AAV (38 men/44 women), median age 61.37 years (24-87 years). Table reflects the main clinical findings and outcomes.

Renal biopsy was performed in 44 patients (36 patients showing a pauci-immune crescentic glomerulonephritis). The remaining 38 patients were not biopsied due to patient disagreement, mild renal disease or contraindication for biopsy.

The most frequent ANCA antibody specificity was MPO (64.6%) followed by PR3 (26.8%) and double positivity in 3 patients (2 MPO and PR3 and 1 MPO and MBG).

43 patients were classified as MPA (52.4%), of those, 18 patients (21.9%) had renal limited vasculitis, 27 GPA (32.9%), and 4 EGPA (4.9%). The rest of the patients had other renal disease (5 microhematuria, 1 amyloidosis, 1 diabetes nephropathy and 1 nephroangiosclerosis).

Nephritic syndrome was the most common renal manifestation when the vasculitis was fully established (56.1%).

The most frequent therapies used were corticosteroids (68.3%), Cyclophosphamide (46.3%), Azathioprine (35.4%), Rituximab (34.1%) and mycophenolate mofetil (23.2%).

After a median follow-up of 6.23 years (7 days-22.9 years) the last median creatinine and glomerular filtration rate (GFR) was 1.4 mg/dl and 43 ml/min, respectively.

Renal function worsened compared to baseline in 78.3% of MPO positive and only in 33% of PR3 positive (p=0.005).

During the first 12 months follow-up, stabilization or normalization GFR (median GFR at 1 year: 50 ml/min) was observed in 47.1% with no statistical differences among ANCA groups (p=0.303).

Renal outcome at the end of follow-up was poorer in MPA than GPA or EGPA (76.3%, 47.4% and 50% respectively worsened GFR from baseline).

16 patients (19.5%) needed dialysis at any moment. Total remission was achieved in 25 patients (30.5%) while relapses were observed in 17 patients (20.7%).

Severe infectious was the main severe side-effect, reported in 26 patients (31.7%).

Conclusion: Most AAV patients had some grade of renal disease during follow-up and almost 20% ended up needing dialysis. Therefore this multi-systemic disease should be managed in a multidisciplinary way to establish an early diagnosis and adequate treatment, limiting chronic disease.

Supporting image 1

Table. Main clinical findings and outcomes of patients with AAV and renal disease.

Abbreviations: ANCA-associated vasculitis (AAV), Granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyarteritis (MPA)


Disclosures: S. Al Fazazi: None; F. Benavides: None; V. Calvo Río: None; m. rodriguez Vidriales: None; c. escagedo Cagigas: None; m. renuncio garcia: None; l. martin penagos: None; R. Blanco: AbbVie, 5, 6, Amgen, 6, AstraZeneca, 2, BMS, 6, Eli Lilly, 6, Galapagos, 2, 6, Janssen, 2, 6, MSD, 6, Novartis, 2, 6, Pfizer, 2, 6, Roche, 5, 6, Sanofi, 6.

To cite this abstract in AMA style:

Al Fazazi S, Benavides F, Calvo Río V, rodriguez Vidriales m, escagedo Cagigas c, renuncio garcia m, martin penagos l, Blanco R. ANCA-associated Vasculitis and Renal Disease in a Multidisciplinary Outpatient Clinic in Northern Spain [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/anca-associated-vasculitis-and-renal-disease-in-a-multidisciplinary-outpatient-clinic-in-northern-spain/. Accessed .
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