title: Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients.
abstract: BACKGROUND
  Fibrillary glomerulonephritis (GN) is a rare disorder with poor renal prognosis. Therapeutic strategies, particularly the use of immunosuppressive drugs, are debated.
  
  
  METHODS
  Case series.
  
  
  METHODS
  27 adults with fibrillary GN referred to 15 nephrology departments in France between 1990 and 2011 were included. All patients were given renin-angiotensin system blockers and 13 received immunosuppressive therapy, including rituximab (7 patients) and cyclophosphamide (3 patients).
  
  
  METHODS
  Clinical and histologic features of patients and kidney disease outcome. Renal response was defined as a >50% decrease in 24-hour proteinuria with <15% decline in estimated glomerular filtration rate (eGFR).
  
  
  RESULTS
  All patients presented with proteinuria, associated with nephrotic syndrome (41%), hematuria (73%), and hypertension (70%). Baseline median eGFR was 49 mL/min/1.73 m(2). Eight patients had a history of autoimmune disease and none had evidence of hematologic malignancy during follow-up. Light microscopic studies showed mesangial GN (70%), predominant pattern of membranous GN (19%), or membranoproliferative GN (11%). By immunofluorescence, immunoglobulin G (IgG) deposits (IgG4, 15/15; IgG1, 9/15) were polyclonal in 25 cases. Serum IgG subclass distribution was normal in the 6 patients tested. After a median 46-month follow-up, renal response occurred in 6 of 13 patients who received immunosuppressive therapy with rituximab (5 patients) or cyclophosphamide (1 patient). Of these, 5 had a mesangial or membranous light microscopic pattern, and median eGFR before therapy was 76 mL/min/1.73 m(2). In contrast, chronic kidney disease progressed in 12 of 14 patients who were not given immunosuppressive therapy, 10 of whom reached end-stage renal disease.
  
  
  CONCLUSIONS
  Number of patients, retrospective study, use of multiple immunosuppressive regimens.
  
  
  CONCLUSIONS
  The therapeutic approach in fibrillary GN remains challenging. The place of immunosuppressive therapy, particularly anti-B-cell agents, needs to be assessed in larger collaborative studies.
authors.affiliation: Department of Nephrology and Renal Transplantation, Centre Hospitalier Universitaire de Poitiers, Université de Poitiers, Poitiers, France; Centre national de référence de l'amylose AL et des autres maladies à dépôt d'immunoglobulines monoclonales, Poitiers, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Javaugue
authors.forename: Alexandre
authors.initials: A
authors.lastname: Karras
authors.forename: François
authors.initials: F
authors.lastname: Glowacki
authors.forename: Brigitte
authors.initials: B
authors.lastname: McGregor
authors.forename: Corinne
authors.initials: C
authors.lastname: Lacombe
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Goujon
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Ragot
authors.forename: Pierre
authors.initials: P
authors.lastname: Aucouturier
authors.forename: Guy
authors.initials: G
authors.lastname: Touchard
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005921:Glomerulonephritis / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012084:Renin-Angiotensin System / Q000187:drug effects*; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Fibrillary glomerulonephritis; cyclophosphamide; immunosuppressive drugs; outcomes; rituximab