The role of post-transplantation cyclosporine treatment in the course of cystic fibrosis pulmonary disease: a case report

Abstract

We describe the case of a 44-year-old female cystic fibrosis (CF) patient (R334W/852del22) who presented symptoms of prolonged acute respiratory infections and recurrent episodes of pneumonia. Computed tomography (CT) scan images of the chest showed that the patient presented airway and parenchymal changes throughout both lungs. She also had decreased lung function performances. In March 2004, she underwent live-related donor renal transplant and started an immunosuppressive therapy with cyclosporine. CT scan images taken respectively 2 and 6 years after transplantation documented a progressive significant size reduction of structural lung damages in both lungs and clinical signs and symptoms of improvements.

Th17/Treg Imbalance in Murine Cystic Fibrosis Is Linked to Indoleamine 2,3-Dioxygenase Deficiency but Corrected by Kynurenines

“…Preliminary observation failed to show a consensus binding sequence for NFAT on the IDO promoter, a finding indicating that other components of the Ca 21 signaling pathway are involved. Irrespective of the molecular mechanism, the finding that inhibition of the Ca 21 -calcineurin-NFAT signaling pathway with CsA restored IDO activity is novel and is consistent with the ability of CsA to inhibit Th17-cell activation (57) and to improve clinical signs and symptoms in patients with CF (58). As calcineurin inhibition is also known to impair virulence in A. fumigatus (59), these findings point to the successful manipulation of this pathway to improve outcome in CF patients with aspergillosis.…”