Disease name and synonyms

Invasive aspergillosis

Fungi responsible

A. fumigatus, A. flavus, A. terreus, A. niger and A. nidulans. Other Aspergillus species are able to produce the disease although the frequency is extremely low.

Disease description

Life-threatening; usually silent disease in its early stages.

Frequency and global burden

Over 30 million people are at risk of invasive aspergillosis each year because of corticosteroid or other therapies, and over 300,000 patients develop it annually. Worldwide, at least 125,000 of these cases are in COPD.

Underlying problems and at risk patients

The disease is common in people with acute leukaemia, stem cell and other transplants (especially lung).

Less commonly, invasive aspergillosis occurs in people with chronic obstructive pulmonary disease, medical intensive care, autoimmune disorders (such as systemic lupus erythematosus), liver cirrhosis, severe burns. However, as some of these diseases are more prevalent than hematological cancer and transplanted patients, the number of individuals with invasive aspergillosis is much higher.

Diagnostic testing

The key tests are CT scans, biopsy for histology (if possible), Aspergillus antigen and/or PCR on blood (leukaemia and stem cell transplant) or sputum or bronchoalveolar lavage. Microscopy  for hyphae and culture are insensitive and slow  No one test can confirm the diagnosis of invasive aspergillosis and different tests perform differentially in different patient groups.

Treatments

Voriconazole is the most effective agent, with alternatives including amphotericin B (liposomal or lipid formulations), micafungin, caspofungin, posaconazole or itraconazole. Flucytosine has a limited role.
IDSA Clinical Practice Guidelines 2016.

Outlook and prognosis

Early diagnosis and prompt treatment is often successful but unfortunately the diagnosis is frequently made late and patients may die. The status of the underlying disease is important in recovery, as continuing immunodeficiency is problematic. Without treatment the mortality rate exceeds 99%. The best outcomes are in leukaemia patients (30%), but outcomes are worse in other blood malignancies and immunodeficiencies.