Pulmonary Complications of Cystic Fibrosis

Bryan Garcia, MD; Patrick A. Flume, MD


Semin Respir Crit Care Med. 2019;40(6):804-809. 

In This Article


CF clinicians define hemoptysis by quantity, and is typically classified as scant (blood-streaked sputum), mild (>5 mL), or massive (>240 mL in 1 day or 2 days of greater than 100 mL). Hemoptysis is the result of chronic local and systemic inflammation leading to localized destruction of the airway epithelium resulting in the development of bronchiectasis and increased growth factors promoting the development of bronchial arterial hypertrophy leading to enlarged, tortuous, and dilated vessels with resultant rupture into the airway.

The incidence and prevalence of hemoptysis in the CF population is poorly defined with previous reports ranging broadly, including 9.1% over a 5-year period, while other reports suggested as high as 21 to 31% over periods as short as 6 months.[4,5,17] Using placebo groups from prior randomized control trials, a rate of 8% of patients reported hemoptysis during an average period of 8 months.[18] Risk factors for the development of hemoptysis overlap with pneumothorax and includes worse lung function, older age, as well as the presence of Pseudomonas in sputum cultures.

The impact of hemoptysis on progression of lung disease is unknown, but massive hemoptysis is a life-threatening emergency. Among CF patients, massive hemoptysis has an average annual incidence of 0.87%, (1 in 115 patients/year). Although massive hemoptysis is more common in patients with severe obstructive lung disease, a prior report described 22% of patients as having normal or mild obstruction based on spirometry. The median age for this complication is 23 years and 75% of patients will be older than 18 years of age.[19,20] Similar to pneumothorax, presence of massive hemoptysis portends a poor prognosis with an attributable risk for mortality between 5.8 and 16.1%. Thus patients with massive hemoptysis warrant consideration for lung transplantation referral.[11,21]

Management of hemoptysis is similar to that of a usual pulmonary exacerbation and includes systemic antibiotics. The modalities of mechanical airway clearance utilized are dependent upon the severity of the bleed (Table 4). When mild hemoptysis is present, continued use of the full armamentarium of airway clearance therapies is recommended. In massive hemoptysis, a temporary holding of airway clearance therapies so as to not cause cough clearance of a formed clot is necessary.[7] In this scenario, airway clearance is typically reinstituted in a stepwise fashion once bleeding has resolved, but there are no guidelines regarding the precise timing and order of reinstitution (Table 5). In conjunction with treatment as described above, assessment to identify and correct any underlying coagulopathy is necessary.[7,20]

For patients with massive hemoptysis or severe instability, bronchial artery embolization (BAE) is the preferred method of achieving resolution of an airway bleed. Although the source of bleed may be identified by computed tomography (CT) scan of the chest or by direct visualization by bronchoscopy, these diagnostic procedures, which may be of greater utility in other lung pathologies, are not recommended in CF due to low clinical yield and increased delay in definitive management.[7] Despite successful initial embolization, recurrence is common. Patients experiencing massive hemoptysis requiring BAE have increased mortality from respiratory failure and referral for evaluation of lung transplantation is appropriate.[7,22,23] Complications of embolization range from mild including headache and chest pain to severe including the development of pulmonary hypertension, stroke, and permanent paralysis from spinal artery infarction.