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Aspergillosis

Orpha number ORPHA1163
Prevalence of rare diseases Unknown
Inheritance
  • Sporadic
Age of onset Variable
ICD 10 code
  • B44
MIM number -
Synonym(s) -

Summary

Aspergillosis covers a large spectrum of fungal diseases that primarily affect the lungs and are caused by members of the genus Aspergillus. A. fumigatus seems to be the most frequent species. The transmission of fungal spores to the human host is via inhalation. The clinical manifestations depend upon the immunological state of the patient, and may include hypersensitivity reactions (allergic bronchopulmonary aspergillosis (ABPA)), noninvasive colonization of previously damaged tissue (pulmonary aspergilloma), acute or chronic limited invasive disease (chronic necrotizing pulmonary aspergillosis (CNPA)) or rapidly progressive invasive disease (invasive aspergillosis (IA)). ABPA occurs in conjunction with asthma and cystic fibrosis. CNPA is a sub-acute process most commonly associated with underlying lung disease, alcoholism, or chronic corticosteroid therapy. Aspergilloma is a fungal ball that develops in existing cavitary lung lesions. IA is an often fatal infection that occurs in severely immunosuppressed patients, and is characterized by fungal invasion of blood vessels. Dissemination to other organs may occur. The incidence of IA was reported to vary between 3 and 7% in bone marrow transplant (BMT) patients, between 1.5 and 4% in liver transplant recipients, and to be approximately 10% in lung transplant recipients, and 14% in patients with onco-hematological diseases and cardiac transplant recipients. Diagnosis is based on histopathological findings and immunodetection of specific antigens. Prophylaxis consists of isolating high-risk patients in laminar air flow (LAF) rooms. Voriconazole, itraconazole, the investigational azoles (posaconazole, ravuconazole, anidulafungin and micafungin) with anti-mould activity, and amphotericin B all possess a reasonably broad spectrum of activity against Aspergillus. Despite advances in therapy, the invasive forms of aspergillosis are often associated with significant morbidity and mortality. *Author: Dr J. Garbino (February 2005)*.

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