Chest
Volume 134, Issue 4, October 2008, Pages 815-823
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Recent Advances in Chest Medicine
Bronchiectasis

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Bronchiectasis, which was once thought to be an orphan disease, is now being recognized with increasing frequency around the world. Patients with bronchiectasis have chronic cough and sputum production, and bacterial infections develop in them that result in the loss of lung function. Bronchiectasis occurs in patients across the spectrum of age and gender, but the highest prevalence is in older women. The diagnosis of bronchiectasis is made by high-resolution CT scans. Bronchiectasis, which can be focal or diffuse, may occur without antecedent disease but is often a complication of previous lung infection or injury or is due to underlying systemic illnesses. Patients with bronchiectasis may have predisposing congenital disease, immune disorders, or inflammatory disease. The treatment of bronchiectasis is multimodality, and includes therapy with antibiotics, antiinflammatory agents, and airway clearance. Resectional surgery and lung transplantation are rarely required. The prognosis for patients with bronchiectasis is variable given the heterogeneous nature of the disease. A tailored, patient-focused approach is needed to optimally evaluate and treat individuals with bronchiectasis.

Section snippets

Pathophysiology

Bronchiectasis is the anatomic distortion of conducting airways that results in chronic cough, sputum production, and recurrent infections8 (Fig 1). Regardless of the underlying cause, bronchiectasis results when inflammatory and infectious damage to the bronchial and bronchiolar walls leads to a vicious cycle of airway injury.4, 8, 9, 10 The recurrence or persistence of airway infection and inflammation results in airway damage that leads to further infection, a spiraling cycle of infection

Prevalence

Based on a review of an insurance claim database, it is estimated that at least 110,000 persons in the United States are currently being treated for non-CF bronchiectasis.9, 18 Weycker et al18 reported a prevalence in the United States of 4.2 per 100,000 persons aged 18 to 34 years and 272 per 100,000 persons among those ≥ 75 years of age. Bronchiectasis is being recognized with increasing frequency because of the widespread use of high-resolution chest CT (HRCT) scanning.19 In addition, there

Diagnosis

Bronchiectasis should be suspected in patients who present with chronic cough productive of mucopurulent sputum. Occasionally, a dry nonproductive cough is the manifesting symptom. Other symptoms of bronchiectasis include dyspnea, hemoptysis, and nonspecific constitutional complaints like fatigue and weight loss. Bronchiectasis is more common in women than in men and, though seen across the age spectrum, is more frequently encountered in middle-aged and elderly persons.18 Physical findings in

Natural History

The clinical course of non-CF bronchiectasis is variable. Some patients have few to no symptoms, and others have daily symptoms and progressive loss of lung function. One large clinical trial30 showed a frequency of 1.5 exacerbations per year in patients from North America, the United Kingdom, and Ireland who were receiving “usual” care for their bronchiectasis. In the past few years, two studies5, 31 have demonstrated a decline of approximately 50 mL/yr in FEV1 in patients with non-CF

Etiologies

Establishing the cause of bronchiectasis may be difficult. Even with exhaustive clinical, laboratory, and pathologic testing, up to 50 to 80% of cases of bronchiectasis may still be idiopathic.21, 34, 35, 36 Several cohorts of US and UK bronchiectasis patients have been characterized (Table 1). Nicotra et al34 reviewed 123 patients with bronchiectasis who were seen at the University of Texas Health Center at Tyler and found that they were predominantly white, female nonsmokers in the sixth

Microbiology

Nonenteric Gram-negative bacteria commonly infect areas of bronchiectasis, though Staphylococcus aureus and NTM are also commonly encountered as well (see Table 2 for a summary of the bacteriology of bronchiectasis). About one third of patients with bronchiectasis are chronically colonized with P aeruginosa. Patients with P aeruginosa experience an accelerated decline in lung function and more frequent exacerbations.31 Patients with no pathogens isolated from their sputum had the mildest

Treatment

The goals of bronchiectasis treatment are to reduce the number of exacerbations and to improve quality of life (Table 3). If an underlying systemic etiology is identified and is treatable, then it should be addressed. For example, Ig replacement for documented deficiency or steroid therapy for ABPA are indicated, although it is unclear whether those interventions alter the natural history of bronchiectasis. Antimicrobial therapies should be aimed at identified pathogens; hence, sputum cultures

Antimicrobial Therapy

The role of the use of maintenance antibiotic therapy is uncertain in patients with non-CF bronchiectasis. Rotating oral antibiotic strategies have been commonly used but without evidence from controlled trials. A retrospective report53 of 26 patients with bronchiectasis who were treated with cycles of alternating antibiotics, including a quinolone, showed radiographic stability of disease in 77% of patients; the length of therapy was from 6 to 84 months. An older study54 of 10 patients who had

Reduction of Airway Inflammation

Therapy with inhaled corticosteroids and oral macrolides may reduce airway inflammation in patients with bronchiectasis. Tsang et al62 demonstrated that therapy with inhaled fluticasone reduced sputum levels of inflammatory markers, and they have subsequently published a 12-month clinical trial63 that showed clinical improvement in patients who had been treated with 500 μg of inhaled fluticasone twice per day compared to placebo. Of note, therapy with systemic steroids has never been studied in

Mobilization of Airway Secretions

Pharmacologic agents and the mechanical mobilization of secretions have been evaluated to a limited degree in patients with non-CF bronchiectasis. Short-acting or long-acting bronchodilator adrenergic and anticholinergic agents are commonly prescribed, but there have been no randomized controlled trials to support their use.66, 67 The mucolytic agent, recombinant human DNase I, had adverse effects when studied in patients with non-CF bronchiectasis and hence should not be used as a maintenance

Surgery

Resectional surgery for the treatment of bronchiectasis may be considered in those patients with focal disease, in those who do not respond to conventional management, and in those with uncontrolled hemoptysis despite the use of interventional radiology techniques. The complete resection of bronchiectasis was reported75 in 118 of 143 young patients with bronchiectasis (mean age, 23.4 years) with a 23% morbidity rate and a 1.3% mortality rate. Successful localized resection has been reported76

Approach to the Patient With Bronchiectasis

Patients in whom bronchiectasis has been diagnosed should be evaluated for potential underlying causes. Patients with focal disease may require bronchoscopy to evaluate for a localized airway obstruction as the cause of the bronchiectasis.78 Rarely, acute pneumonia can result in “pseudobronchiectasis,” so patients need to undergo an HRCT scan when they are clinically stable.79 Patients with diffuse bronchiectasis should be assessed for underlying systemic abnormalities including congenital

Conclusions

Bronchiectasis, which was once thought to be decreasing in prevalence, is now resurging in the developed world and continues to be a common respiratory disease in areas of the world in which people have less access to health care. Clinicians need to be vigilant for patients with bronchiectasis, so that a tailored clinical evaluation can be performed to detect underlying causes and an appropriate multimodality treatment plan can be initiated.

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    The author has been on advisory boards for Gilead Sciences Inc, Pharmaxis Inc, and Transave Inc, and has received grant funding (in the past) from Pathogenesis Inc and Genentech Inc.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

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