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

Cindy Marques (1, 2, 3, 4, 4) , Julien Carvelli (5, 6, 7, 8, 9) , Lucie Biard (10, 11, 12) , Stanislas Faguer (13, 14) , François Provôt (15) , Marie Matignon (16) , Jean-Jacques Boffa (17, 3) , Emmanuelle Plaisier (17, 3) , Alexandre Hertig (17, 3) , Maxime Touzot (18) , Olivier Moranne (19, 20) , Xavier Belenfant , Djillali Annane (21) , Thomas Quéméneur , Jacques Cadranel (2, 3, 17) , Hassan Izzedine , Nicolas Bréchot (4) , Patrice Cacoub (4, 2, 1, 3, 4) , Alexis Piedrafita (13, 13) , Noemie Jourde-Chiche (5, 7, 8, 6) , David Saadoun (1, 2, 4, 3, 4)

1 I3 - Immunologie - Immunopathologie - Immunothérapie [CHU Pitié Salpêtrière]
2 CHU Saint-Antoine [AP-HP]
3 SU - Sorbonne Université
4 CHU Pitié-Salpêtrière [AP-HP]
5 LA CONCEPTION - Hôpital de la Conception [CHU - APHM]
6 Centre de néphrologie et transplantation rénale [Hôpital de la Conception - APHM]
7 C2VN - Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research
8 APHM - Assistance Publique - Hôpitaux de Marseille
9 AMU - Aix Marseille Université
10 SBIM - Service de biostatistique et information médicale de l’hôpital Saint Louis (Equipe ECSTRA)
11 CRESS (U1153 / UMR_A_1125 / UMR_S_1153) - Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité
12 Hôpital Saint-Louis
13 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
14 CIC 1436 - Centre d'investigation clinique de Toulouse
15 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
16 Groupe Henri Mondor-Albert Chenevier
17 CHU Tenon [AP-HP]
18 AURA Paris - Plaisance
19 CHU Nîmes - Centre Hospitalier Universitaire de Nîmes
20 AIDMP - Aide à la Décision pour une Médecine Personnalisé - Laboratoire de Biostatistique, Epidémiologie et Recherche Clinique - EA 2415
21 Hôpital Raymond Poincaré [AP-HP]

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.

Mots clés: glomerulonephritis; anti-glomerular basement membrane disease; outcome; mortality; Goodpasture's disease; vasculitis

Domaines: Immunologie; Urologie et Néphrologie; Santé publique et épidémiologie

Cindy Marques, Julien Carvelli, Lucie Biard, Stanislas Faguer, François Provôt, et al.. Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients. Frontiers in Immunology, 2019, 10, pp.1665. ⟨10.3389/fimmu.2019.01665⟩. ⟨hal-02282561⟩