title: Rituximab in anti-GBM disease: A retrospective study of 8 patients.
abstract: OBJECTIVE
  Anti-glomerular basement membrane (GBM) disease is a rare autoantibody-mediated disorder presenting as rapidly progressive glomerulonephritis, and often with pulmonary hemorrhage. Antibody removal with plasmapheresis and immunosuppressive drugs are the cornerstones of the treatment. Data regarding the use of specific B-cell depleting therapy such as rituximab are lacking.
  
  
  METHODS
  We conducted a retrospective observational study of 8 patients with severe and/or refractory GBM disease that received rituximab therapy.
  
  
  RESULTS
  Eight patients (2 men, 6 women) with a mean age of 26 ± 13.1 years old were included. Seven had severe renal involvement [median creatinin level was 282 μmol/l, range (65-423)] requiring high immunosuppressive or plasmapheresis dependent, and two had relapse of pulmonary hemorrhage including one with renal failure. Patients received an initial immunosuppressive treatment including steroid and cyclosphosphamide (n = 8) and plasmapheresis (n = 5). Except one late relapse, rituximab therapy was started within two months after diagnosis. All patients except one received 4 weekly dose of rituximab (375 mg(2)). Anti-GBM antibodies were still present in 6/8 patients, at rituximab initiation. Complete remission was observed in 7 out of 8 patients, mostly 3 months after rituximab therapy. After a mean follow-up of 25.6 months (range 4-93), patient and renal survival were 100% and 75% respectively, but rituximab use did not improve GFR. Anti-GBM antibodies remained negative for all patients during follow-up. Only one patient developed a severe bacterial infection but no opportunistic or viral infections were reported.
  
  
  CONCLUSIONS
  Rituximab may represent an additional and/or alternative therapy in the induction treatment of anti-GBM disease.
authors.affiliation: Service de néphrologie, IFRNT, Le Kremlin-Bicêtre, France. Electronic address: mtouzot@gmail.com.
authors.forename: Maxime
authors.initials: M
authors.lastname: Touzot
authors.affiliation: Département de Médecine Interne et d'Immunologie clinique, Groupe Hospitalier Pitié-Salpêtrière, 84, boulevard de l'Hôpital, Paris 75013, France.
authors.forename: Johanne
authors.initials: J
authors.lastname: Poisson
authors.affiliation: Département de Néphrologie et Transplantation d'Organes, Centre Hospitalo-Universitaire de Rangueil, Toulouse, France.
authors.forename: Stanislas
authors.initials: S
authors.lastname: Faguer
authors.affiliation: Département de Néphrologie et Transplantation d'Organes, Centre Hospitalo-Universitaire de Rangueil, Toulouse, France.
authors.forename: David
authors.initials: D
authors.lastname: Ribes
authors.affiliation: Service de Médecine interne, groupe hospitalier Cochin, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.affiliation: Service de médecine Interne CH lisieux, France.
authors.forename: Loic
authors.initials: L
authors.lastname: Geffray
authors.affiliation: Service de réanimation médicale, CHU Bicêtre, Le Kremlin-Bicêtre, France.
authors.forename: Nadia
authors.initials: N
authors.lastname: Anguel
authors.affiliation: Service de néphrologie, IFRNT, Le Kremlin-Bicêtre, France; Institut National de la Santé et de la Recherche Médicale INSERM U1014 Villejuif, France.
authors.forename: Helene
authors.initials: H
authors.lastname: François
authors.affiliation: Service de néphrologie, hôpital européen Georges-Pompidou, Paris, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Karras
authors.affiliation: Département de Médecine Interne et d'Immunologie clinique, Groupe Hospitalier Pitié-Salpêtrière, 84, boulevard de l'Hôpital, Paris 75013, France; Centre national de référence maladies systémiques et autoimmunes rares, DHU Inflammation, Immunopathologie, Biothérapie, Université Paris VI-Pierre et Marie Curie, Paris, France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.affiliation: Service de néphrologie, IFRNT, Le Kremlin-Bicêtre, France; Institut National de la Santé et de la Recherche Médicale INSERM U1014 Villejuif, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Durrbach
authors.affiliation: Département de Médecine Interne et d'Immunologie clinique, Groupe Hospitalier Pitié-Salpêtrière, 84, boulevard de l'Hôpital, Paris 75013, France; Centre national de référence maladies systémiques et autoimmunes rares, DHU Inflammation, Immunopathologie, Biothérapie, Université Paris VI-Pierre et Marie Curie, Paris, France. Electronic address: david.saadoun@pls.aphp.fr.
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D001323:Autoantibodies / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D008212:Lymphocyte Depletion / Q000379:methods; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; C051951:antiglomerular basement membrane antibody; D000069283:Rituximab
keywords: Goodpasture syndrome; Immunosuppression; Rituximab; Treatment