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

Laura Massella, Andrea Onetti Muda, Antonia Legato, Giacomo Di Zazzo, Kostas Giannakakis, Francesco Emma

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)1269-1275
Number of pages7
JournalPediatric Nephrology
Issue number7
Publication statusPublished - Jul 2010


  • Alport syndrome
  • Angiotensin converting enzyme inhibitors
  • Angiotensin receptor blockers
  • Cyclosporine A
  • Glomerular basement membrane
  • Hereditary nephritis

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health


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