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Management of CF Lung Disease

Emerging Pathogens

Allergic Bronchopulmonary Aspergillosis (ABPA)

Aspergillus is a fungus. There are several species that may exist in the airways of patients with CF without notice. However, some patients develop an allergic reaction to Aspergillus (ABPA) and it can lead to lung inflammation and further scarring and fibrosis. It may also pose a serious threat to patients who have received organ transplants.

Aspergilli are abundant in soil and water, and preventive measures include reducing exposure to construction, gardening, and lawn cutting. Water leaks that have not dried within 72 hours may also be a source.

ABPA is difficult to diagnose. Treatment involves antifungal medication and corticosteroids to reduce inflammation. It appears that suppressive antibiotic therapy may present a risk factor.⁴³

Nontuberculous Mycobacteria (NTM)

There are several strains of mycobacteria that have been identified in sputum samples of patients with CF. Particularly alarming is Mycobacterium abscessus, because clinicians are finding it “relentlessly” progressive and extremely difficult tor eat. NTM infection results in rapid lung deterioration.

NTM is also found in patients in association with ABPA, and is thought to be due to specific host immune factors. ABPA is treated with corticosteroids, and although steroids have not been shown to be a predisposing factor for NTM in the past, they may play a role in further suppressing the lungs’ defences.

Several studies have now uncovered perplexing NTM infection in patients with CF. The consensus is for close surveillance of patients with unexplained lung deterioration, especially in the presence of ABPA or those patients receiving steroid therapy.⁴⁴

References:
43. Saiman L, Siegel J. Infection control in cystic fibrosis. Clin Microbiology Reviews 2004 January;17(1):57-71.
44. Mussaffi H, Rivlin J, Shalit I, Ephros M, Blau H. Nontuberculous mycobacteria in cystic fibrosis associated with allergic bronchopulmonary aspergillosis and steroid therapy. Eur Respir J 2005;25(2):324-8.