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

Frontiers in Immunology

卷:10

Hassan Izzedine¹  Marie Matignon²  Emmanuelle Plaisier³  Jean-Jacques Boffa³  Alexandre Hertig³  Maxime Touzot⁴  Olivier Moranne⁵  Xavier Belenfant⁶  Djillali Annane⁷  Thomas Quéméneur⁸  Jacques Cadranel⁹  Nicolas Bréchot¹⁰  Patrice Cacoub¹²  David Saadoun¹²  Julien Carvelli¹³  Alexis Piedrafita¹⁵  Cindy Marques¹⁵  Lucie Biard¹⁶  François Provôt¹⁷  Noémie Jourde-Chiche¹⁸  Stanislas Faguer¹⁸ 

[1] Inflammation-Immunopathology-Biotherapy Department (DHU i2B) and Sorbonne Université, Paris, France;

[2] 0Department of Nephrology and Renal Transplantation, Groupe Hospitalier Henri-Mondor, AP-HP, Créteil, France;

[3] 1Sorbonne Université, UPMC Université Paris 06, Hôpital Tenon, Urgences Néphrologiques et Transplantation Rénale, Paris, France;

[4] 2AURA Paris Plaisance, Paris, France;

[5] 3Service Néphrologie-Dialyses-Aphérèse, Hôpital Caremeau, CHU Nîmes, et Faculté de Médecine Université de Montpellier-nimes, Nîmes, France;

[6] 4Nephrology and Dialysis, Centre Hospitalier Intercommunal André Grégoire, Montreuil, France;

[7] 5General ICU, Hôpital Raymond Poincaré, AP-HP, Garches, France;

[8] 6Department of Internal Medicine, Centre Hospitalier, Valenciennes, France;

[9] 7Chest Department and Constitutive Center for Rare Pulmonary Disease, Hôpital Tenon, AP-HP;

[10] 8Department of Nephrology, Peupliers Private Hospital, Ramsay Générale de Santé, Paris, France;

[11] 9Medical-Surgical Intensive Care Unit, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France;

[12] AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, Paris, France;

[13] Aix-Marseille Univ, APHM, C2VN, INRA 1260, INSERM 1263, CHU de la Conception, Centre de Néphrologie et Transplantation Rénale, Marseille, France;

[14] CNRS, FRE3632, Paris, France;

[15] Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose, Paris, France;

[16] Department of Biostatistics and Medical Information, INSERM UMR1153 ECSTRRA Team, Hôpital Saint Louis, AP-HP, Paris, France;

[17] Department of Nephrology, Centre Hospitalier Régional Universitaire de Lille, Lille, France;

[18] Département de Néphrologie et Transplantation d'organes, Centre de référence des maladies rénales rares, Hôpital Rangueil, CHU de Toulouse, Toulouse, France;

[19] INSERM, UMR_S 959, Paris, France;

[20] Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Sorbonne Université, UPMC Univ Paris 06, UMR 7211, Paris, France;

关键词: anti-glomerular basement membrane disease;    Goodpasture's disease;    glomerulonephritis;    vasculitis;    outcome;    mortality;   

DOI  :  10.3389/fimmu.2019.01665

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown