title: High-dose intravenous immunoglobulin pulse therapy in patients with progressive immunoglobulin A nephropathy: a long-term follow-up.
abstract: In progressive immunoglobulin A nephropathy (IgAN), intravenous immunoglobulin (IVIg) treatment has been used to delay disease progression, but the long-term efficacy is largely unknown. We report the clinical outcomes after IVIg therapy in six male patients with progressive IgAN [median glomerular filtration rate (GFR) 31 ml/min per 1.73 m(2)] followed for a median observation period of 8 years. In this single-arm, non-randomized study, IVIg was given monthly at a dose of 2 g/kg body weight for 6 months. The course of renal function was assessed by linear regression analysis of GFR and proteinuria, and was compared to eight patients with IgAN (median GFR 29 ml/min per 1.73 m(2)) without IVIg as a contemporaneous control group. IgAN disease progression was delayed after IVIg therapy on average for 3 years. The mean loss of renal function decreased from -1.05 ml/min per month to -0.15 ml/min per month (P = 0.024) and proteinuria decreased from 2.4 g/l to 1.0 g/l (P = 0.015). The primary end-point (GFR < 10 ml/min or relapse) occurred 5.2 years (median; range 0.4-8.8) after the first IVIg pulse, and after 1.3 years (median; range 0.8-2.4) in the control group (P = 0.043). In Kaplan-Meier analysis, the median renal survival time with IVIg was prolonged by 3.5 years (IVIg 4.7 years versus control 1.2 years; P = 0.006). IVIg pulse therapy may be considered as a treatment option to reduce the loss of renal function and improve proteinuria in patients with progressive IgAN.
authors.affiliation: Division of Nephrology, Department of Internal Medicine I, Ulm University Hospital, Ulm, Germany.
authors.forename: F M
authors.initials: FM
authors.lastname: Rasche
authors.forename: F
authors.initials: F
authors.lastname: Keller
authors.forename: P M
authors.initials: PM
authors.lastname: Lepper
authors.forename: C
authors.initials: C
authors.lastname: Aymanns
authors.forename: W
authors.initials: W
authors.lastname: Karges
authors.forename: L-C
authors.initials: LC
authors.lastname: Sailer
authors.forename: L von
authors.initials: Lv
authors.lastname: Müller
authors.forename: D
authors.initials: D
authors.lastname: Czock
mesh_terms: D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D001794:Blood Pressure; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate; D005922:Glomerulonephritis, IGA / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007116:Immunization, Passive / Q000009:adverse effects / Q000379:methods*; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D011507:Proteinuria / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors