Diagnosis

Complications of Lung Disease

Pneumothorax

Pneumothorax is associated with chest pain, shortness of breath, and hemoptysis, which is the coughing of blood from the respiratory tract. It occurs with CF and its incidence increases with age.¹

Bronchiectasis

Bronchiectasis develops with repeated infections and inflammation.

Some patients also progress to develop hemoptysis, usually in the form of blood-streaked sputum. In approximately 1% of patients with CF, hemoptysis becomes serious, usually with an acute exacerbation. If it becomes life-threatening, surgical intervention is needed.^1,3

Digital Clubbing

As lung disease advances, patients can develop digital clubbing. Though not a pulmonary complication, per se, digital clubbing develops in most patients with CF.

The continuous decline in lung function decreases the amount of oxygen reaching the extremities. As a result, the fingers and toes become clubbed or wide and round at the tips, in nearly all patients with cystic fibrosis. The nail beds also turn bluish.^1,3

Aspergillosis

While more than 50% of patients with CF display antibodies to the fungus Aspergillus fumigatus, a small number develop an allergic reaction to the antigen. Between 2% and 11% of CF patients develop allergic bronchopulmonary aspergillosis (ABPA).

Expectoration of rust-brown sputum plugs is a symptom of the condition. ABPA causes inflammation in the lungs, which may lead to further scarring and fibrosis.^3,30

References:
1. Boucher RC. Cystic Fibrosis. In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL et al., editors. Harrison's Internal Medicine. 16th ed. New York: McGraw-Hill; 2005. p. 1-9.
3. Welsh M. Cystic Fibrosis. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 22nd ed. Philadelphia: WB Saunders; 2004. p. 1-22.
30. Cystic Fibrosis Foundation. Allergic Bronchopulmonary Aspergillosis. University of Wisconsin Hospital and Clinics 2005 July;1-2. Accessed February 28, 2007.