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Am J Kidney Dis. 2013 Oct;62(4):679-90. doi: 10.1053/j.ajkd.2013.03.031. Epub 2013 Jun 4.

Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients.

Author information

1
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.

Abstract

BACKGROUND:

Fibrillary glomerulonephritis (GN) is a rare disorder with poor renal prognosis. Therapeutic strategies, particularly the use of immunosuppressive drugs, are debated.

STUDY DESIGN:

Case series.

SETTING & PARTICIPANTS:

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).

OUTCOMES & MEASUREMENTS:

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.

LIMITATIONS:

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.

KEYWORDS:

Fibrillary glomerulonephritis; cyclophosphamide; immunosuppressive drugs; outcomes; rituximab

PMID:
23759297
DOI:
10.1053/j.ajkd.2013.03.031
[Indexed for MEDLINE]

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