Exacerbations of cystic fibrosis lung disease

Respiratory symptoms such as cough and increased sputum are the most common presenting features of a cystic fibrosis (CF) exacerbation. Abnormal respiratory signs (eg tachypnoea, increased work of breathing, crackles, wheeze) may be present. Nonrespiratory signs and symptoms include loss of appetite, weight and energy; occasionally, an exacerbation presents with only nonrespiratory features.

Pulmonary function may be reduced and new infiltrates may be present on the chest X-ray.

Approximately 10% of patients with CF have coexisting allergic bronchopulmonary aspergillosis (ABPA). Differentiating between ABPA and a CF exacerbation can be difficult; see ABPA for more information.

Management of exacerbations is usually directed by the patient’s CF management plan and undertaken in consultation with a specialist CF centre.

Respiratory infection should be treated promptly at the onset of signs or symptoms, even if chest examination is normal. If symptoms are relatively mild, treatment may begin with a course of oral antibiotics. If there is no improvement after 2 to 4 weeks, a course of intravenous antibiotic treatment is given. Inhaled antibiotics may sometimes be used either as an adjunct to oral or intravenous antibiotics, or alone.