title: Sequential therapy with cyclophosphamide and mycophenolic acid in patients with progressive immunoglobulin A nephropathy: a long-term follow-up.
abstract: In progressive immunoglobulin (Ig)A nephropathy (IgAN), cyclophosphamide pulse therapy (CyP), high-dose intravenous immunoglobulins (IVIg) and mycophenolic acid (MPA) have been used to stop progressive loss of renal function, but disease progression may occur after the end of the initial treatment. Here, we report the long-term follow-up of patients with progressive IgAN with MPA as maintenance therapy after CyP (CyP-MPA). In a median observation time of 6·2 years, we analysed the slopes of the loss of renal function of 47 patients with biopsy-proven IgAN and treated with CyP. Thirty-one patients with further progression were treated with MPA maintenance for a median time of 5·2 years. Follow-up was compared with symptomatic therapy and IVIg as historically matched control groups. Median loss of renal function was reduced significantly from 0·9 ml/min to 0·1 ml/min per month with CyP (P < 0·05), and with MPA in patients with a relapse from -0·4 ml/min to -0·1 ml/min per month (P < 0·05) until the end of the study. Proteinuria decreased significantly from 1·6 g/l to 1·0 g/l after CyP, and during MPA treatment to 0·6 g/l (P = 0·001 Friedman test). Median renal survival time was in patients with CyP 10·5 years (range = 3·2-17·8), with CyP-MPA 10·7 years (range = 8·3-13·1), with IVIg 4·7 years (range = 2·6-6·6), and in untreated patients 1·2 years (range = 0·8-1·6; log-rank test P < 0·01). In patients with progressive IgAN, our long-term follow-up observation indicates that sequential CyP-MPA therapy maintains renal survival significantly.
authors.affiliation: Department of Internal Medicine, Neurology, Dermatology, Clinic for Endocrinology, Nephrology, Section of Nephrology, University Leipzig, Leipzig, Germany.
authors.forename: F M
authors.initials: FM
authors.lastname: Rasche
authors.affiliation: Department of Internal Medicine I, Division of Nephrology, University Hospital of Ulm, Ulm, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Keller
authors.affiliation: Department of Head Medicine and Oral Health, Department of Ophthalmology, University Leipzig, Leipzig, Germany.
authors.forename: W G
authors.initials: WG
authors.lastname: Rasche
authors.affiliation: Center for Geriatric Medicine at Bezirksklinikum Regensburg, Department of Psychiatry and Psychotherapy, University Regensburg, Regensburg, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Schiekofer
authors.affiliation: Department of Diagnostic and Interventional Radiology, University Leipzig, Leipzig, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Kahn
authors.affiliation: Department of Diagnostic and Interventional Radiology, University Leipzig, Leipzig, Germany.
authors.forename: J
authors.initials: J
authors.lastname: Fahnert
mesh_terms: D000328:Adult; D000368:Aged; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D018450:Disease Progression; D005500:Follow-Up Studies; D005922:Glomerulonephritis, IGA / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000187:drug effects / Q000502:physiology; D060046:Maintenance Chemotherapy; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D011507:Proteinuria / Q000188:drug therapy; D012307:Risk Factors
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D009173:Mycophenolic Acid
keywords: IgA nephropathy; cyclophosphamide; immunosuppression; long-term follow-up; mycophenolic acid