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antibodies to Aspergillus. Treatment consists of a prolonged course of oral
steroids (prednisone or prednisolone), usually starting at 2 mg/kg/day, with
subsequent taper and close follow-up. IgE levels should be followed at regular
intervals both as indication of response to therapy and as a warning of reexacerbation. There are no current studies to suggest a clear benefit of antifungal
therapy along with steroids, although some physicians use oral itraconazole
therapy as it may help shorten the course of oral steroids.
Pulmonary Embolism. There is no current literature to suggest there is an
increased incidence of pulmonary embolism (PE) in children with CF. However,
it should be considered in the differential diagnosis if there is acute onset of chest
pain, shortness of breath, and tachypnea. Because of chronic changes seen on
CXR and CT with chronic lung disease, interpretation of imaging may be
challenging to unequivocally confirm or refute PE. The risks of anticoagulation or
thrombolytic therapy for patients with more than mild pulmonary disease are not
trivial, considering the propensity of CF patients to have hemoptysis.
Pleuritis. Pleuritic chest pain can occur in CF patients during acute or subacute
bacterial exacerbations or acute viral infections. The pain usually improves with
oral analgesia and antibiotic treatment if bacterial exacerbation is suspected.
Gastroesophageal Reflux Disease. While many patients with CF take acid
suppression medications (e.g., H2 blocker or protein pump inhibitor [PPI]) for
enhancement of exogenous pancreatic enzyme function, the incidence of GERD
in children with CF is as high as 55% in some studies. Acute exacerbations of
GERD can cause symptoms of gastritis and esophagitis including significant
chest pain in the epigastrium and retrosternal regions. Medications, such as
NSAIDs, recent dietary changes, stress, and ethanol may exacerbate GERD. An
empiric trial of increased acid control may be warranted, but all patients with
recurrent symptoms of GERD, including regurgitation and chest pain, should be
followed closely after ED discharge. In refractory cases, referral to a
gastroenterologist for a formal evaluation is appropriate to determine need for
upper GI series, pH/impedance probe study, and/or endoscopy.
Other Causes of Chest Pain. Chest pain is a common complaint in patients with
CF and can stem from a variety of underlying processes ( Table 99.5 ).

Chest pain of cardiac origin is rare in the pediatric CF population. While
cardiac pain is more common outside the pediatric patient age group, the rare
pediatric CF patient with severe pulmonary disease, nonpulmonary pain, and