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Cyclosporine A treatment in patients with Alport syndrome: a single-center experience

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Abstract

Limited and discordant data are available on cyclosporine A (CsA) treatment for proteinuria in Alport syndrome (AS). To address this lack of consistent data, we have studied 15 AS patients (14 males; mean age 15.3 ± 6.0 years) treated with CsA. Patient selection criteria included a urinary protein/creatinine ratio ≥1 mg/mg and a creatinine clearance >40 ml/min/1.73 m2. CsA treatment was started at an initial dose of 5 mg/kg/day and subsequently adjusted to reach target C2 levels of 500 ng/ml. Renal function, proteinuria, and blood pressure were monitored. Blood pressure was treated to avoid the administration of angiotensin converting enzyme or angiotensin receptor blockers for the first 2 years of therapy. The average follow-up was 3.5 years. Five patients had chronic renal failure at the beginning of treatment, of whom three and one reached end-stage renal failure within 1 and 3 years, respectively. In the remaining 11 patients, the glomerular filtration rate declined by 11 ± 6% within 6 months, but remained stable thereafter. Proteinuria decreased by 63 ± 21% from baseline, but returned nearly to baseline after 2.5 years of follow-up. Based on these results, we suggest that CsA is effective in reducing proteinuria in patients with Alport syndrome but that this effect is temporary. Our data do not support the use of CsA therapy for proteinuric patients with AS, particularly if they have chronic renal failure.

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Authors and Affiliations

  1. Department of Nephrology and Urology, Bambino Gesù Children’s Hospital and Research Institute, Nephrology and Dialysis Unit, Piazza S. Onofrio 4, 00165, Rome, Italy

    Laura Massella, Antonia Legato, Giacomo Di Zazzo & Francesco Emma

  2. Campus Bio-Medico University, Pathology Unit, Rome, Italy

    Andrea Onetti Muda

  3. Department of Pathology and Internal Medicine, La Sapienza University, Rome, Italy

    Kostas Giannakakis

Authors
  1. Laura Massella
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  2. Andrea Onetti Muda
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  3. Antonia Legato
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  4. Giacomo Di Zazzo
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  5. Kostas Giannakakis
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  6. Francesco Emma
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Correspondence to Laura Massella.

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Massella, L., Muda, A.O., Legato, A. et al. Cyclosporine A treatment in patients with Alport syndrome: a single-center experience. Pediatr Nephrol 25, 1269–1275 (2010). https://doi.org/10.1007/s00467-010-1484-3

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  • DOI: https://doi.org/10.1007/s00467-010-1484-3

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