Disease name and synonyms

Severe asthma with fungal sensitization (SAFS), Fungal-associated (severe) asthma

Fungi responsible 

Many, some alone, some collectively. Aspergillus fumigatus, Penicillium chrysogenum, Cladosporium herbarum, Alternaria alternata, Candida albicans, Trichophyton spp. and probably others. 

Disease description

Severe asthma as defined by the 2009 ATS/ERS guidelines or earlier BTS guidelines. In practice this means a low FEV1 or peak flow (usually persistently), high dose inhaled steroids and/or frequent courses of oral steroids. (See video of patient sensitive to alternaria).

Frequency and global burden

Probably worldwide. Severe asthma affects 5-20% of those with asthma, depending on definition and denominator. Of these, 35-50% have SAFS, depending on how extensively they are tested. Six million of people with SAFS  are a conservative estimate.

Underlying problems and at risk patients

Asthma. No specific risk factors identified. Possibly genetic in part.

Diagnostic testing

Total IgE may be normal or elevated, but is <1,000 KIU/L. Skin test or specific IgE test positive for any fungus.

Treatments

Conventional treatment for asthma. Itraconazole (or another azole, not fluconazole) benefits ~60% patients in terms of quality of life, although not necessarily improved lung function.

Outlook and prognosis

Severe asthma is a very debilitating disorder, with frequent medical contacts and multiple treatments. Poorly treated or late treatment results in some intensive care admissions or deaths each year, but it not known how many of these are in SAFS patients.