versão impressa ISSN 0102-3586
J. Pneumologia v.29 n.5 São Paulo set./out. 2003
Bronchiectasis: diagnostic and therapeutic features: A study of 170 patients*
José da Silva MoreiraI; Nelson da Silva PortoII; José de Jesus Peixoto CamargoIII (te-sbct); José Carlos FelicettiIV; Paulo Francisco Guerreiro CardosoV (te-sbct); Ana Luiza Schneider MoreiraVI; Cristiano Feijó AndradeVII
IProfessor of Internal Medicine
IIMedical Director of the Department of Radiology
IIIHead of the Department of Thoracic Surgery Specialist, as designated by the Brazilian Society of Thoracic Surgery
VThoracic Surgeon Specialist, as designated by the Brazilian Society of Thoracic Surgery
VIPhysician of Internal Medicine
VIIAssociate Surgeon, Department of Thoracic Surgery
BACKGROUND: Bronchiectasis is a disease frequently seen by physicians in Brazil
and leads to significant morbidity and lower quality of life for the affected
OBJECTIVES: To study diagnostic and therapeutic aspects in a series of bronchiectasis patients admitted to a hospital specializing in pulmonary diseases.
METHODS: Signs, symptoms and therapeutic results, as well as microbiological and radiographic data, were studied in 170 patients hospitalized between 1978 and 2001: 62.4% female and 37.6% male; age range: 12 to 88 (mean: 36.8). In 52.5% of the patients, there was a history of childhood pneumonia. Tuberculosis was detected in 19.8% and 8.8% had bronchial asthma. Kartagener syndrome was found in 2 patients.
RESULTS: The most common symptoms were cough (100%), expectoration (96%) and rales (66%). Pulmonary lesions were unilateral in 46.5% of the cases. Pneumococcus, H. influenzae or mixed flora was found in 85% of the sputa examined. All 170 patients received antibiotics and were subjected to postural drainage, and 88 (younger patients with higher functional reserves) were also submitted to pulmonary resections (82 unilateral and 6 bilateral). Among the clinically treated patients, there were 2 deaths and multiple hospitalizations were more frequent. Follow up of the surgically treated patients showed that most presented significant symptom improvement and few required readmission.
CONCLUSIONS: In the majority of the patients undergoing lung resection surgery, chronic bronchopulmonary symptoms were either attenuated or completely cured, in contrast to the less favorable outcomes seen in patients receiving only clinical treatment
Key words: Bronchiectasis/diagnosis. Bronchiectasis/therapy. Bronchiectasis/surgery. Bronchiectasis/complications. Computed Tomography. X-ray /methods. Inpatients. Retrospective studies.
List of abbreviations used in this article:
COPD Chronic obstructive pulmonary disease
CT Computed tomography
FEV1 Forced expiratory volume in the first second
Bronchiectasis generally arises from episodes of bronchiolitis or bronchitis that took place during a childhood viral or bacterial infection, or when such episodes are associated with other conditions, such as cystic fibrosis or ciliary dyskinesia, that propitiate infections.(1-3) Under such circumstances, the small lateral bronchi are obliterated, and the larger, more permeable bronchi are consequently dilated.(4,6) The inflammatory process is perpetuated, and mediated, in part by cytokines, through the liberation of substances derived mainly from neutrophils.(7-9) The inflammatory process, together with the processes of reparation and fibrosis, causes the dilation to become definitive, leading to clinical manifestations such as cough, purulent expectoration and hemoptysis, reflecting the tendency of the bronchi to retain secretions. Secondary infection and the appearance of intense vascularization in the dilated bronchi follow.(10) Moist crackles are frequently heard and digital clubbing is observed in up to a third of all cases.(11) However, in most patients, respiratory impairment is not usually severe.(12) Respiratory failure and significant abnormalities in ventilation or oxygenation are uncommon, except in cases in which the pulmonary lesions are extensive or bilateral or in which there is significant co-morbidity, such as chronic obstructive pulmonary disease (COPD).(13)
Computed tomography (CT) has progressively been replacing bronchography in the investigation of bronchiectasis, and can show even very limited dilation, as well as increases in cross-sectional area and wall thickness of the bronchi.(14,15) An important criterion used in deciding the grade and meaning of bronchial dilation is size in comparison with the diameter of the adjacent pulmonary artery branch. This is possible with the cross-sectional views obtained from CT, especially high resolution CT.(16) Both methods (CT and bronchography) are useful in determining the dimensions of the bronchiectasis in patients who are candidates for surgery.(17)
Physiotherapeutic maneuvers (postural drainage in particular)(18) and broad-spectrum antibiotics such as amoxicillin and other members of the tetracycline family, as well as first and second generation cephalosporins, are used in the clinical treatment of bronchiectasis. Bronchiectasis is commonly caused by infection with pneumococcal bacteria, Haemophilus influenzae or, occasionally, with Moraxella catarrhalis or anaerobes.(19 Additional antibiotics, such as quinolones, aminoglycosides, semi-synthetic penicillin or vancomycin, may sometimes be necessary, especially in patients with cystic fibrosis, in whom P. aeruginosa and S. aureus are frequently found.(20) There have been reports of infections with Mycobacterium avium, in which treatment was required, particularly in white women above 60 years of age who presented bronchiectasis in the middle lobe and lingula. (21) Lung resection is usually only indicated in patients with localized, unilateral and symptomatic bronchiectasis who have adequate lung function and present severe suppuration or hemoptysis.(22,23) Comparative analysis between clinical treatment and surgical treatment in patients who had similar degrees of disease has demonstrated that surgically treated patients have a longer life expectancy and better quality of life.(22) Conversely, the life expectancy of patients with bronchiectasis who receive clinical treatment is lower than that of the general population. This difference is more significant after 60 years of age, when an age-related decline in immunocompetence makes people more vulnerable to infections. In light of these observations and owing to advances in surgical treatment, which have allowed low morbidity interventions, every patient with bronchiectasis should, in principle, be considered a candidate for surgery. Obviously, each case should be considered individually, and the factors that may worsen the prognosis or prevent the procedure should be carefully weighed.(17,24) Surgery may be indicated even in (selected) cases of localized bilateral pulmonary lesions.(25,26)
The objective of this paper is to analyze the diagnostic and therapeutic aspects of 170 cases of bronchiectasis treated and studied on a pulmonary diseases ward, to list the diagnostic particularities of the disease and to evaluate the treatment choices and outcomes.
From 1978 to 2001 in the city of Porto Alegre, RS, 42,500 patients were admitted to a hospital specializing in pulmonary diseases. Of that number, 170 (0,4%) were diagnosed with bronchiectasis. Diagnoses were based on clinical, radiological, bronchographic and (from 1990 onwards) CT data. Patient charts were reviewed at the end of each of the following three periods: 1978-1987; 1988-1992; 1993-2001. New cases during each period were progressively combined with previous ones.(27) During those periods, the methodology used to review cases was similar, using the observations reported on patient charts. The format of the patient charts underwent minor modifications due to advances in techniques, especially imaging techniques. Special attention was paid to the recovery of clinical data, which are frequently prejudiced in retrospective revisions. Medical teams, most composed of various observers, analyzed all of the patients involved. However, only one investigator was employed in the coordination and revision of case data.
Of the 170 patients, 106 (62.4%) were female and 64 (37.6%) were male, ages ranged from 12 to 88 (mean: 36.8), and 40 (23.5%) were smokers. In 52.5% of the patients, there was a history of childhood pneumonia, 19.8% had previously been treated for tuberculosis, and 8.8% had been treated for bronchial asthma. Kartagener Syndrome was diagnosed in 2 adult patients (approximately 1.2%). Patients with cystic fibrosis or acquired immunodeficiency syndrome were excluded from analysis.
Clinical manifestations, as well as radiographic, functional and microbiological findings, were assessed and the therapeutic measures (clinical or surgical) were analyzed. There was no correlation between the type of bronchiectasis (cylindrical or cystic) and the therapeutic measures taken.
In the comparison of the cases treated surgically with those treated clinically, continuous variable age was analyzed using t-test and the remaining non-parametric variables were analyzed using the chi-square test for proportions. The adopted level of significance was 5%.
Table 1 lists the clinical manifestations described in the history or verified by the physician at the time of hospitalization for the 170 patients studied. Most symptoms, especially coughing and expectoration, had persisted years and, in more than 80% of the cases, were traced back to childhood.
Clinical treatment (antibiotics, postural drainage and supportive measures) alone was used in 82 patients (49.2%) and surgical treatment (lung resection) was used in 88 (51.8%). The patients who received surgical treatment were, in general, younger, had had symptoms for less time, had smaller lesions (typically unilateral) and had better lung function. Table 2 summarizes the findings for both groups.
All the patients initially received antibiotics, primarily penicillin, amoxicillin and first- and second-generation cephalosporins, and were submitted to respiratory physiotherapy with postural drainage. The 82 patients who received clinical treatment continued to receive this regimen during hospitalization or at home after discharge. Of those 82, 3 presented severe hemoptysis and were submitted to bronchial artery embolization. Among the clinically treated patients who were hospitalized, 2 died due to chronic cor pulmonale. Of the 88 patients who were submitted to surgery, 82 (93.2%) underwent unilateral resection and 6 (6.8%) were given bilateral resections (Table 3).
The 6 patients who were submitted to bilateral resection had good functional reserve (FEV1 greater then 70% of the predicted value), and their lesions, which were resected, were severe, symptomatic and located in restricted areas of both lungs.
A significant increase in bronchopulmonary secretion was observed in the immediate post-operative period. Major complications, however, were seen only in 12 (13.6%) of the patients: pneumonia (in 5), pneumothorax (in 3), empyema (in 2) and intrapleural coagulation (in 2). No deaths occurred among the patients who underwent surgical treatment. Follow-up exams showed that they remained virtually free of bronchopulmonary secretion and infection. Rehospitalization was unnecessary in 84% of surgical patients and only 1 surgical patient was readmitted more than twice in the post-operative period. The patients who were treated clinically were hospitalized several times (2 of them more than 10 times) or frequently had to be seen in the outpatient clinic. Cases of lung transplantation were not included in the present study.
In developing countries, due to the high prevalence of severe childhood respiratory infections, significant numbers of patients still suffer from bronchiectasis.(28) In the present study, in which we evaluated, over a 23-year period, admissions to a hospital specializing in respiratory diseases, we found that bronchiectasis was the primary diagnosis in only 0.4% of cases. This is still a high rate of incidence when compared to that seen in developed countries.(19) In 72.3% of the cases, there was a history of childhood pneumonia or tuberculosis. This is in agreement with data in the literature and confirms local epidemiological data regarding acute respiratory infections, primary tuberculosis and their bronchopulmonary sequelae.(2,29)
The clinical manifestations presented by the 170 studied patients practically all of them with cough and sputum production, and many with hemoptysis are similar to those described in other patient studies.(18) The frequency of digital clubbing (28.3%) is similar to that found in previous case studies, but considerably higher than the 3% observed in developed countries, which is very likely indicative of the lesser degree of severity of bronchiectasis-related respiratory impairment in those countries.(19)
In this study, most patients presented normal respiratory function, especially, as expected, those selected for surgical treatment. The (generally older) patients who received only clinical treatment presented more pronounced functional impairment. They presented a wider variety of symptoms, were readmitted several times, and 2 died during hospitalization. There were no deaths among the patients who underwent surgery. In the past, the mortality rate for patients undergoing surgery to treat bronchiectasis was as high as 12%.(30) In recent studies, however, this mortality rate has been shown now to be approximately 1%.(17,22) In a follow-up study of 842 adult Finnish bronchiectasis patients, long-term mortality (over a period of 8 to 12.9 years) was 28%.(31) However, the present study did not aim to evaluate such long-term outcomes.
Several patients in the present series were hospitalized after their diagnostic work-up had already been partially completed in the outpatient clinic. Symptomatic, and presenting restricted, localized pulmonary lesions, these patients were, in principle, candidates for surgical treatment. In 6 patients who had bilateral lesions but still presented normal respiratory function, surgery was performed on both lungs, resectioning the portions most affected. There have been many advances in the surgical management of patients with bronchiectasis.(17,25,26) Principal among these is the routine use of epidural analgesia, which guarantees effective pain control and allows sequential resections of localized bilateral bronchiectasis to be performed simultaneously, requiring that the patient be anesthetized only once. Additional advances include the use of staples (in place of sutures) to reduce air leakage and to forestall the removal of chest tubes, early and intensive post-operative respiratory physiotherapy and post-operative rehabilitation (including nutritional rehabilitation of undernourished patients). More recently, the resection of more severely affected areas of lung has been performed in selected patients with systemic diseases such as cystic fibrosis.(32)
Embolization of bronchial arteries, which was performed in 3 patients in our series, can be carried out with low morbidity and is quite effective in controlling bleeding, although some patients suffer episodic bleeding weeks or months after the procedure.(33,34)
The data displayed in Table 2 referring to patient age, respiratory function and extent of lung lesions show that the profiles of patients in the clinically treated group and of those in the surgically treated group were dissimilar, which could explain the difference in treatment outcomes. Although the groups were not comparable, there was evident improvement in the conditions of the patients who underwent surgery. Bronchopulmonary symptoms, which were persistent or chronic prior to surgery, disappeared or were significantly attenuated. This is in agreement with the observations of other authors who have compared the outcomes of surgical and clinical treatment in bronchiectasis patients.(17,22,24) Although not addressed in the present study, younger patients with extensive bilateral lung lesions may benefit from bilateral pulmonary transplantation.(35)
The distribution of bacterial flora was similar in all cases (patients with restricted pulmonary lesions and those with more extensive lesions), and pneumococcus, H. influenzae and anaerobes were predominant. In general, these patients came to the hospital directly from their homes, where such bacteria can thrive, and it is well known that these bacteria are the most common sources of respiratory infection in individuals having no significant systemic disease.(3,18,19)
In conclusion, it can be said that bronchiectasis, which affects both genders, is still a serious health problem in southern Brazil. Typically, individuals suffering from bronchiectasis present symptoms from childhood on. Preventative measures should be taken to avoid acute respiratory infections during the early stages of childhood development, and complications, when they occur, should be treated immediately to prevent the onset of bronchiectasis. Bronchiectasis frequently affects young individuals, significantly affecting their quality of life. Therefore, the most effective and definitive therapy should be sought to control the symptoms. Surgical treatment is clearly the optimal choice.
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Dr. José S. Moreira
Pavilhão Pereira Filho (Santa Casa)
Rua Annes Dias, 285
90020-090 Porto Alegre, RS, Brasil
Submitted: 12/01/2003. Accepted after revision: 12/05/2003
* Study performed at the Pavilhão Pereira Filho University Hospital (Irmandade Santa Casa de Misericórdia FFFCMPA, UFRGS Porto Alegre).