Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients.

Cindy Marques,Hassan Izzedine,Alexis Piedrafita,Nicolas Bréchot,Julien Carvelli,Jacques Cadranel,Stanislas Faguer,Alexandre Hertig,Marie Matignon,Olivier Moranne,Xavier Belenfant,Maxime Touzot,Lucie Biard,David Saadoun,Emmanuelle Plaisier,Djillali Annane,Noémie Jourde-Chiche,Patrice François ,Jean‐Jacques Boffa ,P Cacoub ,T Quéméneur

doi:10.3389/fimmu.2019.01665

Abstract

We report the overall and renal outcome in a French nationwide multicenter cohort of 119 patients with anti-glomerular basement membrane (anti-GBM) disease. Sixty-four patients (54%) had an exclusive renal involvement, 7 (6%) an isolated alveolar hemorrhage and 48 (40%) a combined renal and pulmonary involvement. Initial renal replacement therapy (RRT) was required in 78% of patients; 82% received plasmapheresis, 82% cyclophosphamide, and 9% rituximab. ANCA positive (28%) patients were older (70 vs. 47 years, p < 0.0001), less frequently smokers (26 vs. 54%, p = 0.03), and had less pulmonary involvement than ANCA- patients. The 5 years overall survival was 92%. Risk factors of death (n = 11, 9.2%) were age at onset [HR 4.10 per decade (1.89–8.88) p = 0.003], hypertension [HR 19.9 (2.52–157 0.2) p = 0.005], dyslipidemia [HR 11.1 (2.72–45) p = 0.0008], and need for mechanical ventilation [HR 5.20 (1.02–26.4) p = 0.047]. The use of plasmapheresis was associated with better survival [HR 0.29 (0.08–0.98) p = 0.046]. At 3 months, 55 (46%) patients had end-stage renal disease (ESRD) vs. 37 (31%) ESRD-free and 27 (23%) unevaluable with follow-up < 3 months. ESRD patients were older, more frequently female and had a higher serum creatinine level at presentation than those without ESRD. ESRD-free survival was evaluated in patients alive without ESRD at 3 months (n = 37) using a landmark approach. In conclusion, this large French nationwide study identifies prognosis factors of renal and overall survival in anti-GBM patients.

Highlights

Anti-glomerular basement membrane disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs [1]
Anti-GBM disease is a rare disease with an estimated incidence between 0.5 and 1.6 case per million per year [23] but it represents 1 to 5% of all types of glomerulonephritis and ∼20% of rapidly progressive glomerulonephritis [24,25,26]
With previous series [7, 10, 27], we reported a high frequency of antineutrophil cytoplasmic antibodies (ANCA) positive anti-GBM disease patients

Summary

Introduction

Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs [1]. It is an organ-specific autoimmune disease mediated by circulating autoantibodies directed against the non-collagenous domain of the α3 chain of type IV collagen [α3(IV)NC1] [2,3,4,5]. Twenty-one to 47% of patients have antineutrophil cytoplasmic antibodies (ANCA) [6,7,8,9,10] They mostly display anti-myeloperoxidase (MPO) specificity [11, 12] and could be older than those with anti-GBM positivity alone [13], with a male preponderance [9]

Methods

Results

Conclusion