Microbes in Cystic Fibrosis

MRSA
Pseudomonas

Bacteria
Burkholderia Staph
NTM

Influenza Candida

Viruses Fungi
RSV Rhinovirus Aspergillus
Everyone is exposed to fungi
• Every day we breath:
• >20,000 times/day

• We inhale:
• ~10,000 liters of
air/day
>50,000
• 1 cubic meter of air can fungal spores
contain:
Many species of fungi cause disease
Rising awareness of fungi in CF
• Increased life expectancy

• Increased percentage of immunocompromised CF patients

• Detection methods are improving

How often do we find fungi in CF?
• Historically, reported prevalence varies greatly
• Aspergillus spp: 0 to 36%
• Scedosporium spp: 0 to 9%

• Appears to be increasing over time

• Multi center studies are showing a higher prevalence of fungi than CF

registry data
Similar prevalence in recent studies
What are risk factors for fungal colonization
in CF?
• CF lung disease
• Impaired mucus clearance
• Immunogenic dysfunction

• Decreased lung function

• Older age
• Inhaled and oral antibiotic use
• Inhaled steroid use
• Macrolide antibiotics
We found fungi – now what?
• Colonization?

• Allergy?

• Infection?
Aspergillus species
• Over 100 species
• A. fumigatus most common
• Everywhere in the environment
• Soil, water, decomposing material

• Wide spectrum of Aspergillus-associated disease

• Allergic bronchopulmonary aspergillosis (ABPA) is now well described in CF
• What is the role of A. fumigatus in the non-ABPA CF lung?
Wide spectrum aspergillosis
Inhalation of Aspergillus spores

Healthy Lung Asthma/CF lung CF Lung

Efficient Clearance Hypersensitivity Colonization/Infection

No infection APBA Bronchitis & Aspergilloma

ABPA: Minimal diagnostic criteria
• Clinical deterioration
• Total serum IgE > 500 IU/mL
• Immediate cutaneous reactivity to Aspergillus or in
vitro demonstration of IgE antibody to A. fumigatus
• One of the following:
• Precipitins to A. fumigatus  or in vitro demonstration of IgG antibody to A. fumigatus
• New or recent abnormalities on chest radiography (infiltrates or mucus plugging) or
chest CT (bronchiectasis) that have not cleared
Microbes in Cystic Fibrosis
MRSA
Pseudomonas

Bacteria
Burkholderia Staph
NTM

Influenza Candida

Viruses Fungi
RSV Rhinovirus Aspergillus
Everyone is exposed to fungi
• Every day we breath:
• >20,000 times/day

• We inhale:
• ~10,000 liters of
air/day
>50,000
• 1 cubic meter of air can fungal sores
contain:
Many species of fungi cause disease

Romani L. 2004. Nat Rev Immunol.

Rising awareness of fungi in CF
• Increased life expectancy

• Increased percentage of immunocompromised CF patients

• Detection methods are improving

How often do we find fungi in CF?
• Historically, reported prevalence varies greatly
• Aspergillus spp: 0 to 36%
• Scedosporium spp: 0 to 9%

• Appears to be increasing over time

• Multi center studies are showing a higher prevalence of fungi than CF

registry data
We found fungi – now what?
• Colonization?

• Allergy?

• Infection?
Aspergillus species
• Over 100 species
• A. fumigatus most common
• Everywhere in the environment
• Soil, water, decomposing material

• Wide spectrum of Aspergillus-associated disease

• Allergic bronchopulmonary aspergillosis (ABPA) is now well described in CF
• What is the role of A. fumigatus in the non-ABPA CF lung?
Wide spectrum aspergillosis
Inhalation of Aspergillus spores

Healthy Lung Asthma/CF lung CF Lung

Efficient Clearance Hypersensitivity Colonization/Infection

No infection APBA Bronchitis & Aspergilloma

ABPA: Minimal diagnostic criteria
• Clinical deterioration
• Total serum IgE > 500 IU/mL
• Immediate cutaneous reactivity to Aspergillus or in
vitro demonstration of IgE antibody to A. fumigatus
• One of the following:
• Precipitins to A. fumigatus  or in vitro demonstration of IgG antibody to A. fumigatus
• New or recent abnormalities on chest radiography (infiltrates or mucus plugging) or
chest CT (bronchiectasis) that have not cleared