title: Spectrum and Prognosis of Noninfectious Renal Mixed Cryoglobulinemic GN.
abstract: Noninfectious mixed cryoglobulinemic GN (MCGN) has been poorly investigated. We analyzed presentation and outcome of 80 patients with biopsy-proven MCGN, which were identified in the retrospective French CryoVas survey. MCGN was related to primary Sjögren's syndrome in 22.5% of patients and to lymphoproliferative disorders in 28.7% of patients, and was defined as essential in 48.8% of patients. At presentation, hematuria, proteinuria ≥1 g/d, hypertension, and renal failure were observed in 97.4%, 84.8%, 85.3%, and 82.3% of cases, respectively. Mean±eGFR was 39.5±20.4 ml/min per 1.73 m(2) Membranoproliferative GN was the predominant histologic pattern, observed in 89.6% of cases. Renal interstitium inflammatory infiltrates were observed in 50% of cases. First-line treatment consisted of steroids alone (27.6%) or in association with rituximab (21.1%), alkylating agents (36.8%) or a combination of cyclophosphamide and rituximab (10.5%). After a mean follow-up of 49.9±45.5 months, 42.7% of patients relapsed with a renal flare in 75% of cases. At last follow-up, mean eGFR was 50.2±26.1 ml/min per 1.73 m(2)with 9% of patients having reached ESRD; 59% and 50% of patients achieved complete clinical and renal remission, respectively. A rituximab+steroids regimen prevented relapses more effectively than steroids alone or a cyclophosphamide+steroids combination did, but was associated with a higher rate of early death when used as first-line therapy. Severe infections and new-onset B-cell lymphoma occurred in 29.1% and 8.9% of cases, respectively; 24% of patients died. In conclusion, noninfectious MCGN has a poor long-term outcome with severe infections as the main cause of death.
authors.affiliation: Departments of Nephrology-Transplantation, Institut Necker Enfants Malades, Paris, France; Paris Descartes University, Sorbonne Paris Cité, Paris, France;
authors.forename: Mohamad
authors.initials: M
authors.lastname: Zaidan
authors.affiliation: Paris Descartes University, Sorbonne Paris Cité, Paris, France; Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France;
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.affiliation: Department of Nephrology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium;
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Pozdzik
authors.affiliation: Departments of Nephrology and.
authors.forename: Thierry
authors.initials: T
authors.lastname: Frouget
authors.affiliation: Pathology, University Hospital, Ponchaillou, Rennes, France;
authors.forename: Nathalie
authors.initials: N
authors.lastname: Rioux-Leclercq
authors.affiliation: Department of Nephrology, University Hospital, Bordeaux, France;
authors.forename: Christian
authors.initials: C
authors.lastname: Combe
authors.affiliation: Department of Pathology, University Hospital, Bordeaux, France;
authors.forename: Sébastien
authors.initials: S
authors.lastname: Lepreux
authors.affiliation: Departments of Nephrology-Transplantation.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Hummel
authors.affiliation: Pathology, and.
authors.forename: Laure-Hélène
authors.initials: LH
authors.lastname: Noël
authors.affiliation: Departments of Internal Medicine.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Marie
authors.affiliation: Nephrology and.
authors.forename: Bruno
authors.initials: B
authors.lastname: Legallicier
authors.affiliation: Pathology, University Hospital, Rouen, France;
authors.forename: Arnaud
authors.initials: A
authors.lastname: François
authors.affiliation: Department of Nephrology and Organ Transplant, Rangueil Hospital, Toulouse, France;
authors.forename: Antoine
authors.initials: A
authors.lastname: Huart
authors.affiliation: University of Medicine, Lille, France; Regional University Hospital, Lille, Department of Internal Medicine and Clinical Immunology, Lille, France National Referral Center for Rare Systemic Autoimmune Diseases (Systemic Sclerosis), Lille, France; EA2686, Lille Inflammation Research International Center, Lille, France;
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.affiliation: Department of Clinical Immunology and Internal Medicine, La Conception Hospital, Marseille, France;
authors.forename: Gilles
authors.initials: G
authors.lastname: Kaplanski
authors.affiliation: Department of Nephrology, University Hospital, Poitiers, France;
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.affiliation: Department of Nephrology, University Hospital, Valenciennes, France;
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.affiliation: Department of Nephrology, University Hospital, Amiens, France;
authors.forename: Raifah
authors.initials: R
authors.lastname: Makdassi
authors.affiliation: Department of Nephrology, Dialysis, Transplantation, University Hospital, Angers, France;
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Augusto
authors.affiliation: Departments of Pathology and.
authors.forename: Philippe
authors.initials: P
authors.lastname: Rouvier
authors.affiliation: Department of Nephrology, Georges Pompidou European Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France;
authors.forename: Alexandre
authors.initials: A
authors.lastname: Karras
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche_S 1155, Paris, France;
authors.forename: Chantal
authors.initials: C
authors.lastname: Jouanneau
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche_S 1155, Paris, France;
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Verpont
authors.affiliation: Departments of Pathology.
authors.forename: Patrice
authors.initials: P
authors.lastname: Callard
authors.affiliation: Department of Public Health, Saint-Antoine Hospital, Paris, France; Université Pierre et Marie Curie, Paris, France; Institut National de la Santé et de la Recherche Médicale, U1136, Paris, France;
authors.forename: Fabrice
authors.initials: F
authors.lastname: Carrat
authors.affiliation: Adult Hematology, Necker Hospital, Paris, France;
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.affiliation: Neurology, La Pitié-Salpêtrière Hospital, Assistance Publique - Hôpitaux de Paris, Paris, France;
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Léger
authors.affiliation: Department of Rheumatology, Université Paris-Sud; University Hospital, Paris-Sud, Le Kremlin-Bicêtre, France;
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.affiliation: Dermatology, and.
authors.forename: Patricia
authors.initials: P
authors.lastname: Senet
authors.affiliation: Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, Paris, France; Inflammation-Immunopathology-Biotherapy Department, Paris, France Sorbonne Universités, Paris, France; and.
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche_S 1155, Paris, France; Nephrology and Dialysis, Assistance Publique - Hôpitaux de Paris, Tenon Hospital, Paris, France; Sorbonne Universités, Tenon Hospital, Paris, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Ronco
authors.affiliation: Departments of Pathology.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Brochériou
authors.affiliation: Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, Paris, France; Inflammation-Immunopathology-Biotherapy Department, Paris, France Sorbonne Universités, Paris, France; and.
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche_S 1155, Paris, France; Nephrology and Dialysis, Assistance Publique - Hôpitaux de Paris, Tenon Hospital, Paris, France; Sorbonne Universités, Tenon Hospital, Paris, France emmanuelle.plaisier@tnn.aphp.fr.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Plaisier
mesh_terms: D003449:Cryoglobulinemia / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use
chemical_list: D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide
keywords: histopathology,; membranoproliferative GN