학술논문

Clinical and Prognostic Factors in Patients with IgG4-Related Kidney Disease.
Document Type
Academic Journal
Author
Chaba A; Departement of Nephrology-Dialysis-Transplantation, Assistance Publique des Hôpitaux de Paris (AP-HP), Bicêtre University Hospital, Paris-Saclay University, Le Kremlin Bicêtre, France.; Devresse A; Department of Nephrology, Cliniques universitaires Saint-Luc, Bruxelles, Belgium.; Audard V; Nephrology and Renal Transplantation Department, Assistance Publique des Hôpitaux de Paris (AP-HP), Henri Mondor Hospital University, Rare Disease Center « Idiopathic Nephrotic syndrome », Fédération Hospitalo-Universitaire « Innovative therapy for immune disorders, Créteil, France.; Univ Paris Est Créteil, Institut National de la Santé et de la Recherche Médicale (INSERM) U955, Institut Mondor de Recherche Biomédicale (IMRB), Créteil, France.; Boffa JJ; Department of Nephrology, CHU Tenon, Paris, France.; Karras A; Department of Nephrology, CHU HEGP, Paris, France.; Cartery C; Department of Nephrology, CH Valenciennes, Valenciennes, France.; Deltombe C; Institute for Transplantation, Urology and Nephrology (ITUN) Nantes University Hospital, Nantes, France.; Chemouny J; Department of Nephrology, CHU Rennes, Rennes, France.; Contamin C; Department of Internal Medicine, GHM Grenoble, France.; Courivaud C; Department of Nephrology, CHRU Jean Minjoz, Bensançon, France.; Duquennoy S; Department of Nephrology, Fondation AUB Santé Avranches, France.; Garcia H; Department of Nephrology, Hôpitaux Sorbonne Université, Paris, France.; Joly D; Department of Nephrology, CHU Necker, Paris, France.; Goumri N; Department of Nephrology, CH Chartres, Chartres, France.; Hanouna G; Department of Nephrology, CHU Bichat, Paris, France.; Halimi JM; Department of Nephrology, CHU Tours, Tours, France.; Plaisier E; Department of Nephrology, AURA Plaisance, Paris, France.; Hamidou M; Department of Internal Medicine, CHU Nantes, Nantes, France.; Landron C; Department of Internal Medicine, CHU Poitier, Poitier, France.; Launay D; Univ. Lille, Inserm, CHU Lille, Service de Médecine Interne et Immunologie Clinique, Centre de référence des maladies autoimmunes systémiques rares du Nord et Nord-Ouest de France (CeRAINO), U1286-INFINITE-Institute for Translational Research in Inflammation, Lille, France.; Lebas C; Department of Nephrology, CHU Valenciennes, Valenciennes, France.; Legendre M; Department of Nephrology, CHU Dijon, Dijon, France.; Masseau A; Department of Internal Medicine, CHU Nantes, Nantes, France.; Mathian A; Department of Internal Medicine, Hôpital Cochin, APHP, Paris, France.; Mercadal L; Department of Nephrology, Hôpitaux Sorbonne Université, Paris, France.; Morel N; Department of Internal Medicine, Hôpital Cochin, APHP, Paris, France.; Mutinelli-Szymanski P; Department of Nephrology, CHU Tours, Tours, France.; Palat S; Department of Internal Medicine, CHU Limoges, Limoges, France.; Pennaforte JL; Department of Internal Medicine, CH Epernay, Reims, France.; Peraldi MN; Department of Nephrology, CHU Saint Louis, Paris, France.; Pozdzik A; Department of Nephrology, CHU Brugmann, Bruxelles, Belgium.; Schleinitz N; Department of Internal Medicine, CHU Timone, Marseille, France.; Thaunat O; Department of Nephrology, CH Edouart Heriot, Lyon, France.; Titeca-Beauport D; Department of Nephrology, CHU Amiens, Amiens, France.; Mussini C; Departement of Pathology, Assistance Publique des Hôpitaux de Paris (AP-HP), Bicêtre University Hospital, Paris-Saclay University, Le Kremlin Bicêtre, France.; Touati S; Department of Nephrology, CH Pontoise, Pontoise, France.; Prinz E; Department of Nephrology, NHC Strasbourg, France.; Faller AL; Department of Nephrology, Clinique Sainte Anne, Strasbourg, France.; Richter S; Department of Nephrology, Clinique Sainte Anne, Strasbourg, France.; Vilaine E; Department of Nephrology, CHU Ambroise Paré, France.; Ferlicot S; Departement of Pathology, Assistance Publique des Hôpitaux de Paris (AP-HP), Bicêtre University Hospital, Paris-Saclay University, Le Kremlin Bicêtre, France.; Von-Kotze C; Department of Nephrology, ADPC Marseille Michelet, Marseille, France.; Belliere J; Departement of Nephrology, CHU Toulouse, Toulouse, France.; Olagne J; Department of Pathology, NHC Strasbourg, France.; Mesbah R; Department of Nephrology, Hopital Boulogne-sur-mer, Boulogne-sur-mer, France.; Snanoudj R; Departement of Nephrology-Dialysis-Transplantation, Assistance Publique des Hôpitaux de Paris (AP-HP), Bicêtre University Hospital, Paris-Saclay University, Le Kremlin Bicêtre, France.; Nouvier M; Department of Nephrology, CHLS, Lyon, France.; Ebbo M; Department of Internal Medicine, CHU Timone, Marseille, France.; Zaidan M; Departement of Nephrology-Dialysis-Transplantation, Assistance Publique des Hôpitaux de Paris (AP-HP), Bicêtre University Hospital, Paris-Saclay University, Le Kremlin Bicêtre, France.
Source
Publisher: Wolters Kluwer Health Country of Publication: United States NLM ID: 101271570 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1555-905X (Electronic) Linking ISSN: 15559041 NLM ISO Abbreviation: Clin J Am Soc Nephrol Subsets: MEDLINE
Subject
Language
English
Abstract
Background: IgG4-related kidney disease is a major manifestation of IgG4-related disease, a systemic fibroinflammatory disorder. However, the clinical and prognostic kidney-related factors in patients with IgG4-related kidney disease are insufficiently defined.
Methods: We conducted an observational cohort study using data from 35 sites in two European countries. Clinical, biologic, imaging, and histopathologic data; treatment modalities; and outcomes were collected from medical records. Logistic regression was performed to identify the possible factors related to an eGFR ≤30 ml/min per 1.73 m 2 at the last follow-up. Cox proportional hazards model was performed to assess the factors associated with the risk of relapse.
Results: We studied 101 adult patients with IgG4-related disease with a median follow-up of 24 (11-58) months. Of these, 87 (86%) patients were male, and the median age was 68 (57-76) years. Eighty-three (82%) patients had IgG4-related kidney disease confirmed by kidney biopsy, with all biopsies showing tubulointerstitial involvement and 16 showing glomerular lesions. Ninety (89%) patients were treated with corticosteroids, and 18 (18%) patients received rituximab as first-line therapy. At the last follow-up, the eGFR was below 30 ml/min per 1.73 m 2 in 32% of patients; 34 (34%) patients experienced a relapse, while 12 (13%) patients had died. By Cox survival analysis, the number of organs involved (hazard ratio [HR], 1.26; 95% confidence interval [CI], 1.01 to 1.55) and low C3 and C4 concentrations (HR, 2.31; 95% CI, 1.10 to 4.85) were independently associated with a higher risk of relapse, whereas first-line therapy with rituximab was protective (HR, 0.22; 95% CI, 0.06 to 0.78). At their last follow-up, 19 (19%) patients had an eGFR ≤30 ml/min per 1.73 m 2 . Age (odd ratio [OR], 1.11; 95% CI, 1.03 to 1.20), peak serum creatinine (OR, 2.74; 95% CI, 1.71 to 5.47), and serum IgG4 level ≥5 g/L (OR, 4.46; 95% CI, 1.23 to 19.40) were independently predictive for severe CKD.
Conclusions: IgG4-related kidney disease predominantly affected middle-aged men and manifested as tubulointerstitial nephritis with potential glomerular involvement. Complement consumption and the number of organs involved were associated with a higher relapse rate, whereas first-line therapy with rituximab was associated with lower relapse rate. Patients with high serum IgG4 concentrations (≥5 g/L) had more severe kidney disease.
(Copyright © 2023 by the American Society of Nephrology.)