Cystic Fibrosis (CF) patients commonly have pulmonary colonisation with aspergillus. Invasive aspergillosis (IA) is rare in patients with CF and has an extremely high mortality rate. IA after the first year of solid organ transplantation is rare, except for lung transplantation. A 15 year old boy with CF and diabetes had a liver transplantation aged 12 for CF liver disease. Post-transplantation, he took tacrolimus and prednisolone. He was colonised with aspergillus and pseudomonas but had stable lung function (FEV176%). He presented with jaundice and abdominal pain and had multiple courses of iv antibiotics for suspected cholangitis. Liver biopsy suggested de novo autoimmune hepatitis and he received three days of pulsed methylprednisolone followed by high dose prednisolone and mycophenolate mofetil. He was never neutropenic. Three days later he developed type 1 respiratory failure and haemoptysis with CT evidence of extensive, bilateral, rapidly progressive cavitating lung disease. Despite stopping immunosuppressants and 3 weeks treatment with caspofungin and voriconazole, he died after 7 days of chest drainage for tension hydropneumothoraces. Fatal IA can occur in post-liver transplant CF patients following augmentation of immunosuppression.
Full conference title:
15th European Respiratory Society annual conference
- ERS 15th (2005)