Table 1.
Aspergillus -associated disease states
Disease | Clinical syndromes | Patient groups and notes |
---|---|---|
Invasive aspergillosis (IA): Most common species: A. fumigatus, A. flavus, A. niger, A. terreus, A. ustus, A. versicolor |
Pneumonia and systemic disease; CNS disease most common site of dissemination; fungal keratitis | Induction-consolidation chemotherapy for hematologic malignancies and aplasia BM, lung, and heart transplant recipients Patients with graft vs. host disease, recipients of systemic corticosteroid therapy COPD and critically ill ICU patients Fungal keratitis in agricultural workers with corneal abrasions |
Primary immune deficiencies associated with IA | Affected gene | |
Chronic granulomatous disease A. fumigatus and A. nidulans most common IA species in CGD | Pneumonia, deep tissue disease (e.g., osteomyelitis, brain abscess) | Defects in NADPH oxidase (e.g., in p22, p47, X-linked p91 subunits) |
Job’s syndrome (HIES) | Pneumonia, lung disease at sites of prior bacterial (staphylococcal) lung abscess | STAT3 |
MonoMAC syndrome | Pneumonia | GATA2 |
CARD9 deficiency | Extrapulmonary aspergillosis | CARD9 |
Several Congenital Neutropenia | Pneumonia and systemic disease | ELA2, HAX1 |
Leukocyte adhesion deficiency | Pneumonia and systemic disease | CD18 |
Pulmonary alveolar proteinosis | Pneumonia and systemic disease | Defect in GM-CSF signaling and surfactant catabolism |
Chronic pulmonary aspergillosis (includes SAIA, CNPA, CCPA) | Pneumonia in patients with structural and/or cavitary lung disease; no angioinvasion and hyphae confined to preexisting lung cavity | Affects individuals previously treated for TB Aspergilloma refers to a fungus ball, a clump of hyphae in a pulmonary cavity |
Tracheobronchial aspergillosis | Fungal disease of large airways without evidence of lung parenchymal involvement | Most common in lung transplant recipients and ICU patients |
Allergenic disease | ABPA | Patients with underlying asthma and cystic fibrosis have defective fungal clearance and dysregulated immune responses Hypersensitivity to Aspergillus antigens can lead to progressive bronchiectasis |
Extrinsic allergic alveolitis | Typically observed after very high exposure to airborne conidia |
ABPA, allergic bronchopulmonary aspergillosis; BM, bone marrow; CARD9, caspase recruitment domain-containing protein 9; CCPA, chronic cavitary pulmonary aspergillosis; CGD, chronic granulomatous disease; CNPA, chronic necrotizing pulmonary aspergillosis; CNS, central nervous system; GATA2, GATA binding protein 2; GM-CSF, granulocyte-macrophage colony-stimulating factor; HIES, hyper IgE syndrome; LAD, leukocyte adhesion deficiency; SAIA, subacute invasive pulmonary aspergillosis; SCN, severe combined neutropenia; TB, tuberculosis.