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A caucasian female with Cystic Fibrosis required a combined heart and lung transplant at the age of 15. Immunosuppressant therapy was commenced post-transplant. In August 2005, symptomatology and HRCT identified likely multi-focal pulmonary fungal infection. Cultures were negative. Empirical therapy eventually led to symptomatic improvement. This therapy was later discontinued due to the potential interaction with her immunosuppressant drugs. By this stage she had also developed severe chronic rejection; adequate immunosuppression was therefore of profound importance. However, the cessation of antifungal therapy resulted in a progressive return of her symptoms; successive chest x-rays revealed worsening pulmonary lesions. Aspergillus precipitins were positive, consistent with Aspergillus fumigatus as being the causative organism but unfortunately re-initiation of antifungal therapy was unable to prevent her death. Go to images
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| Diagnosis: The diagnosis in this case was made purely through radiological techniques (High Resolution Computed Tomography), which indicated multi-focal cavitating fungal infection of the lungs as being the most likely diagnosis. All cultures were initially negative, despite repeated broncho-alveolar lavage to obtain samples. Resolution of symptoms on commencement of antifungal therapy further strengthened the presumed diagnosis. Only later was Aspergillus fumigatus identified, following the reactivation of the disease on discontinuation of antifungal therapy, with an Aspergillus precipitins giving a titre of 1/8.
Response to antifungal therapy: Initial treatment with a prolonged course of IV Caspofungin and oral Voriconazole showed only a slow response. Subsequent treatment with IV Voriconazole and IV Ambisome given for a total of four weeks resulted in a greater symptomatic improvement, which persisted for some months. On reactivation of the disease, treatment with voriconazole was unable to prevent her death.
Outcome: The patient died shortly after re-commencement of antifungal therapy.
Case kindly provided by Dr Sarah Bailey, SHO, Wythenshawe Hospital, Manchester M23 9LT
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