Management of idiopathic glomerular disease associated with nephrotic syndrome (INS) remains controversial and one of the most complex areas relates to utilization of the drug cyclosporin. This is despite its demonstrated effectiveness in several histologic types of the INS in randomized controlled trials. Cyclosporin is effective in inducing remission of proteinuria in approximately 80% of steroid-sensitive cases of minimal change disease (MCD). Cyclosporin is also effective in both the induction of remission and long-term preservation of renal function in steroid-dependent/-resistant MCD and steroid-resistant focal segmental glomerulosclerosis (FSGS). The overall response rate in FSGS is lower than in MCD, and long-term therapy (>12 months) may be required to both achieve remission and sustain it. Cyclosporin therapy is also of benefit in reducing proteinuria in 70-80% of patients with steroid-resistant membranous nephropathy (MGN). In MGN, the maximum benefit is often delayed compared to MCD (>12 weeks). Cyclosporin is generally well tolerated and safe. The major concern remains the nephrotoxicity, but with careful monitoring of the patient's renal function; minimizing the maintenance dose and utilizing repeat renal biopsy in those receiving long-term therapy, this risk can be minimized. The algorithms have been developed derived from the best evidence in the literature in each of the histologic types to help provide a guide to the integration of cyclosporin into the management of INS for the practicing nephrologist.
Bibliographical noteFunding Information:
The members of the ‘Cyclosporin in idiopathic nephrotic syndrome’ working group include Professor Daniel Cattran (Chair), Department of Medicine, University of Toronto, Toronto, Ontario, Canada Professor Efstathios Alexopoulos, Department of Nephrology, Aristotelian University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece; Dr Gabriel Choukroun, Nephrology Department, Hôpital Sud, CHU Amiens, Amiens, France; Professor Peter F Hoyer, Clinic of Pediatric Nephrology (Director & Chair), University of Essen, Germany; Professor Peter Heering, Department of Medicine III, Solingen General Hospital, University of Cologne, Solingen, Germany; Professor Atholl Johnston, Department of Clinical Pharmacology, Barts and The London, Queen Mary's School of Medicine & Dentistry, London, UK; Professor Alain Meyrier, Department of Nephrology, Hôpital Georges Pompidou and Université Paris-Descartes Medical School, Paris, France; Professor Patrick Nachman, Division of Nephrology, University of North Carolina, Chapel Hill, NC, USA; Professor Claudio Ponticelli, IRCCS Istituto Auxologico Italiano, Milano, Italy; Dr Manuel Praga, Department of Nephrology, Hospital Universitario 12 de Octubre, Madrid, Spain; Professor Takao Saito, Department of Internal Medicine, Fukuoka University School of Medicine, Fukuoka, Japan; Professor Norishige Yoshikawa, Department of Pediatrics, Wakayama Medical University, Wakayama City, Japan. This report has been generated from the ‘Cyclosporin in idiopathic nephrotic syndrome’ recommendations meeting held in London, UK, in March 2005, which involved experts in nephrology and clinical pharmacology. The purpose of the conference was to form a working group to develop recommendations on the clinical role of Neoral® (cyclosporin microemulsion) in the treatment of idiopathic nephrotic syndrome. This conference was supported by an unrestricted educational grant from Novartis Pharma AG. This study was supported by an unrestricted educational grant from Novartis Pharma AG. Thanks to Dr Brocksmith (Brocksmith Scientific Ltd) for supporting the authors in developing the text and Mrs D Bumford (Denise Bumford Ltd) for help in facilitating the workshop and journal submission.
- Clinical practice
- Focal and segmental glomerulosclerosis
- Idiopathic nephrotic syndrome
- Membranous nephropathy
- Minimal change disease