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PubMed1996

IgG subclass deficiencies associated with bronchiectasis.

Only a small number of patients with IgG subclass deficiencies (IgGSD) have been observed to have bronchiectasis. Moreover, in the series of patients with bronchiectasis, IgGSD have not been found at any frequency, and the etiology of bronchiectasis remains unclear in 29 to 49% of cases. Serum concentrations of total IgG, IgA, and IgG subclasses as well as pulmonary function were measured in 65 patients (aged: 10 to 74 yr) with bronchiectasis of unknown etiology. An ELISA test was performed to quantify subclasses 1 through 4 using subclass-specific antihuman monoclonal antibodies. IgG subclass estimation in a healthy population with age-stratified normal ranges was derived from 100 adults, 37 children aged between 10 and 12 yr, and 27 adolescents aged between 13 and 16 yr. Serum concentrations of specific IgG antibodies to Haemophilus influenzae type b capsular polysaccharide (Hib-PRP) were also assayed by an ELISA test in 19 of the patients (10 with IgGSD and nine with non-IgGSD) and in 58 healthy individuals before and 3 wk after immunization with Hib-PRP conjugated to meningococcal outer membrane protein complex (OMPC). Thirty-one patients (48%) had low serum concentrations of one or more IgG subclasses (19 IgG2 deficiencies, 2 IgG3 deficiencies, 3 IgG4 deficiencies, and 7 combined subclass deficiencies). All patients showed increased levels of total IgG, IgG1, and IgA, but this rise was significantly higher in patients without IgGSD. Patients with IgGSD showed impaired antibody response to Hib-PRP compared with patients with non-IgGSD and the control group. IgGSD, particularly IgG2 deficiency, are not an unusual cause of bronchiectasis. Therefore, serum levels of IgG subclasses must be assayed whenever other causes of bronchiectasis have been ruled out.

Adolescent↗

PubMed2005

HLA-C and killer cell immunoglobulin-like receptor genes in idiopathic bronchiectasis.

RATIONALE: In idiopathic bronchiectasis, lung inflammation and chronic bacterial infection lead to progressive lung damage. A possible role for natural killer (NK) cells is suggested by the observation that familial bronchiectasis occurs in a rare group of individuals with impaired HLA class I expression and consequent NK cell dysfunction. OBJECTIVE: Because the HLA-C locus and killer cell immunoglobulin-like receptors (KIRs) are of key importance for NK cell recognition, we analyzed HLA-C/KIR combinations by genotyping patients with idiopathic bronchiectasis. METHODS: Genomic DNA from 96 individuals with idiopathic bronchiectasis and 101 control subjects was analyzed by polymerase chain reaction with sequence-specific primers. High-resolution HLA-C genotyping was performed in addition to KIR analysis. RESULTS: HLA-Cw*03 alleles and, in particular, HLA-C group 1 homozygosity are associated with the presence of bronchiectasis. Analysis of the relationship between HLA-C and KIR genes suggests a shift to activatory NK cell function. CONCLUSION: This is the first demonstration of genetic susceptibility in idiopathic bronchiectasis. The association with HLA-C group 1 homozygosity, and the interplay between HLA-C and KIR genes, argue for a role for NK cells in the progressive lung damage seen in this disease. This will require further investigation using functional studies.

Bronchiectasis↗

PubMed1992

Role of chronic Pseudomonas aeruginosa infection in the development of bronchiectasis.

To understand the role of Pseudomonas aeruginosa infection in the development of bronchiectasis, we investigated by CT the presence of bronchiectasis in two groups of chronic bronchitis patients and in a control group. There were no differences in clinical or laboratory findings between groups A and B. Three observers without any knowledge of these patients reported bronchiectasis on a scale of 0 to 3 and bronchial wall thickness on a scale of 0 to 3 in each lobe of both lungs. Bronchiectasis and wall thickness scores in group A (chronic bronchitis with P aeruginosa infection) were significantly higher than bronchiectasis scores and wall thickness in group B (chronic bronchitis without P aeruginosa infection). Both scores in group B were higher than those in group C (control group). These findings support the idea that chronic P aeruginosa infection plays a role in the development of bronchiectasis.

Adult↗

PubMed1996

Thin-section CT detection of emphysema associated with bronchiectasis and correlation with pulmonary function tests.

PURPOSE: To evaluate, on thin-section CT scans, the prevalence of emphysema in patients with bronchiectasis and to correlate the results of thin-section CT scans with the results of pulmonary function tests, in order to question whether there was a particular functional test profile in this group of patients. PATIENTS AND METHODS: This is a retrospective study including 90 patients having both thin-section CT scans and pulmonary function tests for bronchiectasis. A CT scoring system was established for assessing the airway disease by the severity and extent of bronchiectasis and by the extent of emphysema. CT scans were reviewed independently by two reviewers and final interpretation was obtained by consensus. Results of thin-section CT scans were correlated with results of pulmonary function tests, including FEV1 and FEV1/FVC to assess air-way obstruction, total lung capacity and residual volume to assess air trapping, and diffusing capacity for carbon monoxide/alveolar volume (DCO/VA). RESULTS: CT evidence of emphysema, which was noted in 45% of the patients (n = 41), was mainly localized in the same bronchopulmonary segments as bronchiectasis. The presence of emphysema was in relation to the extent and to the severity of bronchiectasis. Only eight patients with CT evidence of emphysema had functional evidence of emphysema (20%). When comparing the group of patients with CT evidence of emphysema with the group of patients with no CT evidence of emphysema, the group of patients with CT evidence of emphysema had significantly higher airflow obstruction and air trapping, had significantly lesser value of diffusing capacity, but with no decreased gas transfer (DCO/VA > 80%). CONCLUSION: Our series suggests that there is a high prevalence of emphysema in patients with bronchiectasis. Emphysema that was not suggested using pulmonary function tests in most of the cases could explain in part the higher airway obstruction observed in the group of patients with CT evidence of emphysema. This study could support the suggestive notion that emphysema, which was mainly localized in bronchiectatic lobes, could be due to the inflammatory airway process.

Bronchiectasis↗

PubMed1999

Specific antibody response against the 23-valent pneumococcal vaccine in patients with alpha(1)-antitrypsin deficiency with and without bronchiectasis.

OBJECTIVE: To assess the specific antibody response against polyvalent pneumococcal vaccine in patients with alpha(1)-antitrypsin deficiency (AATD) and respiratory infections. DESIGN AND PARTICIPANTS: We investigated specific IgG, IgG1, and IgG2 antibody responses against the 23-valent antipneumococcal vaccine in 18 patients with AATD phenotype PiZZ, 9 of whom had bronchiectasis and 4 a history of recurrent pneumonia, and compared them with a control group of 40 healthy volunteers. INTERVENTIONS: Blood samples were drawn just prior to and 3 weeks after immunization. MEASUREMENTS AND RESULTS: Quantification of specific IgG and its subclasses was performed by an enzyme-linked immunosorbent assay. For patients with AATD, mean increases in specific antipneumococcal titers were 4.7-fold (25 to 75% quartiles, 2.5- to 6.8-fold) for total IgG, 3.2-fold (1.2- to 4.9-fold) for IgG1, and 2.1-fold (1.8- to 3.7-fold) for IgG2. For the control group, the values were 3.3-fold (1.8- to 5.8-fold) for total IgG, 2. 5-fold (1.9- to 3.4-fold) for IgG1, and 3.1-fold (1.9- to 4.5-fold) for IgG2; differences were not significant. Patients with bronchiectasis showed a tendency toward higher levels of IgG subclasses than both control subjects and patients without bronchiectasis; however, there was a tendency toward lower postvaccination serum levels of specific antipneumococcal IgG, IgG1, and IgG2 in patients with bronchiectasis compared with patients without bronchiectasis, but this trend did not reach statistical significance. Three of the four patients with recurrent pneumonia did not show an appropriate IgG2 response. CONCLUSIONS: These results suggest that, as a group, patients with AATD have a preserved antibody response against pneumococcal polysaccharides. Patients with bronchiectasis show a tendency toward a decreased antibody response, even with increased serum levels of most Ig types. Individuals with an impaired IgG2 response seem to be at increased risk of recurrent pneumonia. Considering the pernicious effect of pulmonary infections on these patients and the preserved antibody response in a majority of them, pneumococcal vaccination should be recommended to patients with AATD.

Adult↗

PubMed2000

Distribution of alpha(1)-antitrypsin alleles in patients with bronchiectasis.

STUDY OBJECTIVES: Bronchiectasis has been reported in a few patients with homozygous alpha(1)-antitrypsin (AAT) deficiency, but the distribution of AAT alleles among bronchiectatic patients is not known. PATIENTS AND DESIGN: Two hundred two patients, 104 men and 98 women, with a mean age (SD) of 63.7 +/- 15.4 years, had bronchiectasis diagnosed by CT scan alone (n = 178), bronchography with or without CT scan (n = 17), or radiography alone (n = 7). AAT phenotypes (classified according to the protease inhibitor [PI] system) were determined by isoelectric focusing in blood samples obtained from all patients. Bronchiectasis was primary in 121 cases and secondary in 81 patients. Allele and phenotype frequencies were compared retrospectively between bronchiectatic patients and healthy blood donors living in the same geographic area. RESULTS: The PI phenotype frequencies among patients were the following: MM, 81.18%; MS, 11.88%; MZ, 3.46%; IZ, 0.49%; IM, 0.49%; SS, 1.48%; SZ, 0.49%; and ZZ, 0.49%. The allelic frequencies among patients were the following: M, 89.1%; S, 7.67%; Z, 2.72%; and I, 0.49%. There was no difference in the distribution of alleles or phenotypes either between patients and control subjects or between patients with secondary and primary bronchiectasis. A significant difference was found between bronchiectatic patients with and without coexisting emphysema (p = 0.028). This difference was caused by an overrepresentation of PI*Z alleles in bronchiectatic patients with coexisting emphysema. CONCLUSIONS: Our results do not support a physiopathologic implication of the AAT genes in the development of bronchiectasis. We suggest that bronchiectasis may be a consequence of emphysema in PI*Z patients rather than a primary effect.

Adolescent↗

PubMed2005

Antibody production deficiency with normal IgG levels in bronchiectasis of unknown etiology.

BACKGROUND: No defined cause of bronchiectasis is currently found in approximately 50% of cases. Bronchiectasis is a common long-term complication in patients with primary hypogammaglobulinemia. STUDY OBJECTIVES: To ascertain whether antibody production deficiency with normal total serum IgG levels is associated with bronchiectasis. DESIGN: Antibody response to a pneumococcal unconjugate vaccine and an Haemophilus influenzae type b conjugate vaccine was prospectively studied in all consecutive adult patients with bronchiectasis of unknown etiology who were assessed in our chest outpatient clinic from January 1994 to October 2001. Serum-specific antibodies were measured by enzyme-linked immunosorbent assay, and the results were compared with those obtained in a healthy adult control group. Antibody production deficiency was defined as a failure to respond to either vaccine. RESULTS: One hundred seven patients were included in the study (mean age, 46.3 years). Antibody production deficiency was diagnosed in 12 patients (11%). A significantly higher incidence of otitis media, lower serum IgG2 subclass levels, and lower preimmunization antibody levels to Streptococcus pneumoniae and H influenzae type b were observed in patients with antibody production deficiency. The probability of antibody production deficiency in patients with a history of otitis media was 20%, 26% in those with low IgG2 subclass levels, and 58% in those with both a history of otitis media and low IgG2 subclass levels. CONCLUSIONS: Antibody production deficiency with normal IgG levels may be associated with bronchiectasis, making it advisable to evaluate the antibody response to both the H influenzae and pneumococcal vaccines in patients with bronchiectasis of unknown etiology, particularly in those with a history of otitis media, low IgG2 subclass levels, and low levels of baseline specific antibodies.

Adolescent↗

PubMed2006

Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice guidelines.

BACKGROUND: Bronchiectasis is a condition that is characterized by the permanent dilation of bronchi with destruction of elastic and muscular components of their walls, usually due to acute or chronic infection. The cardinal symptom is a chronic productive cough. METHODS: Review of articles cited in the systematic literature search, along with others found in Ovid MEDLINE and the Cochrane Library (including the Cochrane Database of Systematic Reviews, the Cochrane Controlled Trial Register, and the Database of Abstracts of Reviews of Effectiveness) from 1966 through 2003. RESULTS/CONCLUSIONS: High-resolution CT scanning of the chest is the preferred means of establishing the diagnosis of bronchiectasis. With the increasing use of antibiotics in the treatment of childhood infection in the last several decades, an increasing percentage of patients with bronchiectasis now have an underlying disorder that predisposes them to chronic or recurrent infection. These include cystic fibrosis, common variable immunodeficiency, HIV infection, primary ciliary dyskinesia, allergic bronchopulmonary aspergillosis, and chronic Mycobacterium avium complex infection. A variety of agents have been used to improve cough effectiveness and prevent infectious exacerbations in patients with bronchiectasis, with variable results. Chest physiotherapy offers a modest benefit in increasing sputum volume, but its long-term effectiveness is unknown. Selected patients with localized idiopathic bronchiectasis that causes intolerable symptoms despite maximal medical therapy should be offered treatment with surgery. Patients with exacerbations of bronchiectasis should be given antibiotics, with the choice of agents depending on the likely causative pathogens.

Bronchiectasis↗

PubMed2006

Addition of inhaled tobramycin to ciprofloxacin for acute exacerbations of Pseudomonas aeruginosa infection in adult bronchiectasis.

RATIONALE: Pseudomonas aeruginosa lung infection in patients with bronchiectasis, a chronic airway disease that is characterized by episodes of exacerbation, is associated with more severe disease and a higher utilization of health-care resources. Inhaled tobramycin solution reduces the number of acute exacerbations in patients with cystic fibrosis (CF)-related bronchiectasis with P aeruginosa infection but remains untested in the treatment of exacerbations in patients with non-CF bronchiectasis. OBJECTIVES: This study tested the effect of adding inhaled tobramycin solution to oral ciprofloxacin (Cip) for the treatment of acute exacerbations of non-CF bronchiectasis in patients with P aeruginosa infection. METHODS: A double-blind, randomized, active comparator, parallel-design study conducted at 17 study centers (5 in the United Kingdom, and 12 in the United States) compared 2 weeks of therapy with Cip with either an inhaled tobramycin solution or placebo in 53 adults with known P aeruginosa infection who were having acute exacerbations of bronchiectasis. MEASUREMENTS: Clinical symptoms, pulmonary function, clinical efficacy, and sputum microbiology were investigated prospectively. MAIN RESULTS: An inhaled solution of Cip with tobramycin, compared to placebo, achieved greater microbiological response but no statistically significant difference in clinical efficacy at days 14 or 21. Clinical and microbiological outcomes at the test of cure (ie, the clinical outcome assessment at day 21) were concordant when an inhaled tobramycin solution was added to therapy with Cip and compared to placebo (p = 0.01). Both subject groups had similar overall adverse event rates, but subjects receiving therapy with an inhaled tobramycin solution reported an increased frequency of wheeze (50%; placebo group, 15%). CONCLUSIONS: The addition of an inhaled tobramycin solution to therapy with oral Cip for the treatment of acute exacerbations of bronchiectasis due to P aeruginosa improved microbiological outcome and was concordant with clinical outcome; the inability to demonstrate an additional clinical benefit may have been due to emergent wheeze resulting from treatment.

Administration, Inhalation↗

PubMed1984

Role of computed tomography in the recognition of bronchiectasis.

To assess the accuracy of computed tomography (CT) in the recognition of bronchiectasis, CT was performed in 11 patients with clinical findings strongly suggestive of this diagnosis. In two patients, CT showed extensive bilateral cystic and varicose bronchiectasis, and bronchography was considered unwarranted. In four patients, bilateral, and in five, unilateral bronchograms were obtained. Correlation was made between the CT and bronchographic findings in these 13 lungs. In only six bronchograms did CT give an accurate assessment of the presence and extent of disease. In five, the diagnosis of cylindrical and varicose bronchiectasis was missed on CT. In two, CT suggested the diagnosis of cylindrical bronchiectasis but the bronchogram was normal. It was concluded that CT may be useful in the diagnosis of cystic bronchiectasis, but is unreliable in detecting cylindrical and varicose changes. Bronchography, therefore, remains the definitive method for establishing the diagnosis, extent, and severity of bronchiectasis.

Adult↗

PubMed2003

Demographic, clinical, and radiographic features of bronchiectasis in dogs: 316 cases (1988-2000).

OBJECTIVE: To determine demographic, clinical, and radiographic features of bronchiectasis in dogs. DESIGN: Retrospective study. ANIMALS: 289 dogs identified through the Veterinary Medical Database (VMDB) and 27 dogs examined at the North Carolina State University Veterinary Teaching Hospital. PROCEDURE: Demographic characteristics of dogs identified through the VMDB were compared with characteristics of the entire population of dogs entered in the VMDB. Medical records of dogs examined at the teaching hospital were reviewed; the diagnosis was confirmed through review of thoracic radiographs. RESULTS: Analysis of data from the VMDB indicated that American Cocker Spaniels, West Highland White Terriers, Miniature Poodles, Siberian Huskies, English Springer Spaniels, and dogs > 10 years old had an increased risk of bronchiectasis. Among dogs examined at the teaching hospital, coughing was the most common clinical sign. There was evidence for excessive airway mucus but not hemorrhage. A variety of bacterial organisms were isolated from tracheal wash and bronchoalveolar lavage samples. On thoracic radiographs, cylindrical bronchiectasis, generalized disease, and right cranial lung lobe involvement were most common. Seven of 14 dogs for which follow-up radiographs were available did not have any progression of radiographic lesions. Median duration of clinical signs prior to diagnosis of bronchiectasis was 9 months (range, 1 day to 10 years). Median survival time was 16 months (range, 2 days to 72 months). CONCLUSIONS AND CLINICAL RELEVANCE: Results suggest that despite substantial clinical abnormalities, dogs with bronchiectasis may survive for years. Certain purebred dogs and older dogs may have an increased risk of developing bronchiectasis.

Age Factors↗

PubMedDate not supplied

Bronchiectasis in systemic sclerosis. A study using high resolution computed tomography.

OBJECTIVE: To detect noninvasively the presence of bronchiectasis in patients with systemic sclerosis (SSc), through the use of high resolution chest computed tomography (HRCT). METHODS: Twenty two patients with SSc, of whom 13 with diffuse and 9 with limited disease, besides a complete history, physical and routine laboratory and immunologic profile, were evaluated by pulmonary function testing and HRCT. The chi square test with Yates' correction, the Fisher's exact test, the Fisher's test (F test) and the "t" test were used for statistical analysis of the results. RESULTS: Eleven patients (50.0%) had decreased carbon monoxide diffusing lung capacity (DLCO) and, out of these, four had restrictive lung disease, based on a combined decrease of forced vital capacity (FVC) and total lung capacity (TLC). Another two patients exhibited this pattern without DLCO impairment. HRCT revealed a ground glass picture in 15 patients (68.2%), fibrosis in 9 (40.9%) (of which 5 with ground glass as well), and cylindrical bronchiectasis in 13 (59.1%). Bronchiectasis was more common in diffuse than in limited SSc, and the difference approached but did not reach the level of statistical significance. On the other hand, it was not correlated with either decreased DLCO, presence of ground glass and fibrosis, or with patients' age and disease duration. CONCLUSION: Although the number of patients included in our study is relatively small, our data, for the first time in the literature, indicate a significant association between scleroderma and bronchiectasis. Bronchiectasis should be included in the list of pulmonary manifestations of SSc, and SSc in the list of conditions causing bronchiectasis.

Adult↗

PubMedDate not supplied

Clinical spectrum of bronchiectasis in children.

The prevalence of bronchiectasis decreased due to the effective use of vaccines and advances of antibiotic therapy after 1970. However, it remains an important long-term morbidity of lower respiratory tract infection in developing country. To evaluate the clinical features of bronchiectasis in a tertiary hospital, we collected 21 patients with a diagnosis of bronchiectasis in Taipei Veterans General Hospital from May, 1984 to Dec, 2001 in pediatric outpatient with the admission of age below 18 years old. The diagnosis was based on the history of recurrent cough with fetid sputum, hemoptysis, or recurrent lobar pneumonia for months at least and radiological findings of lobar infiltration, tram-track like patterns, bronchiolar dilatation or honeycomb patterns. The diagnostic examinations included chest plain radiography, bronchography and chest computed tomography (CT) scans. Respiratory tract infections were the commonest cause predisposing to bronchiectasis in our study. Tuberculosis is not rare in this study. In recent years, immunodeficiency disorders have been recognized. Most patients suffered from recurrent cough and fetid sputum for years before diagnosis was established. Hemoptysis was the second common symptom in our study. The plain chest radiograph of bronchiectasis revealed dilatation of bronchial trees with honeycomb pattern or infiltration only. In recent years, chest CT became the most accurate and being noninvasive diagnostic tool. The initial treatment was primarily medical conservative therapy. Only five patients in our cases underwent pulmonary resection due to persistent hemoptysis, recurrent bacterial pneumonia or pulmonary parenchyma destruction. Most patients still suffered from recurrent pneumonia or occasional exacerbation in the long-term follow-up. In conclusion, bronchiectasis is not uncommon in pediatric population in northern Taiwan. The history of recurrent cough with fetid sputum, hemoptysis, or recurrent pneumonia were the most important clues to early diagnosis of this disease. Early diagnosis and appropriate treatment are effective in order to prevent lung abscess, empyema and pneumothorax, bronchopleural fistula, hemoptysis or cor pulmonale.

Bronchiectasis↗

PubMed2004

Clinical analysis of bronchiectasis in Taiwanese children.

BACKGROUND: The clinical features and etiology of bronchiectasis have rarely been described in non-Caucasian populations, of whom the prevalence of cystic fibrosis is low. In this report, we studied the clinical features of bronchiectasis in Taiwanese children. METHODS: Using a retrospective chart review, 29 cases of bronchiectasis were diagnosed from 1991 through 2001. For each case, the diagnosis was confirmed using high-resolution computed tomography. Medical records were analyzed for demographic data, clinical presentation, spirometric data, and microbial isolation. Radiographic findings were reviewed, and possible causes of bronchiectasis were also identified. RESULTS: There were 17 girls and 12 boys enrolled. Persistent cough, daily sputum production, and hemoptysis were common presenting symptoms. Crackles and wheezing were the most frequent findings during the physical examination. Previous lower airway infection, asthma, and primary immunodeficiency were the most common, but 31.0% of the cases had unknown causes. Dependent lobes were involved more frequently. Simultaneous sinusitis was noted in 70.6% of the cases. Spirometry showed mild airway obstruction in most of the cases. Most specimens (52.2%) from lower airway secretions yielded bacterial pathogens, most commonly Pseudomonas aeruginosa, Haemophilus influenzae and Streptococcus pneumoniae. CONCLUSION: Continuing post-infectious inflammatory changes remains the most important cause of bronchiectasis in a non-Caucasian pediatric population in Northern Taiwan in the 1990s. More than two thirds of these patients had underlying predisposing factors including asthma, immunodeficiency, and swallowing dysfunction. Chronic productive purulent respiratory secretions and persistent crackles should raise the possibility of bronchiectasis in children even in Taiwan where the incidence of cystic fibrosis is low.

Adolescent↗

PubMed1992

High prevalence of asthma in patients with bronchiectasis in Hong Kong.

Eighty five Chinese patients with diffuse or localized bronchiectasis (non-cystic fibrosis) were studied regarding the prevalence of asthma. Twenty three of the 85 had concomitant asthma, diagnosed by history and reversibility on lung function testing either spontaneously or after bronchodilator. None fulfilled the diagnostic criteria of allergic bronchopulmonary aspergillosis (ABPA). Asthma preceded the onset of bronchiectasis in 13 patients and developed after long duration of bronchiectasis in seven, while the temporal onset could not be differentiated in three patients. Patients with both asthma and bronchiectasis had inferior spirometric values, higher prevalence of bronchial hyperresponsiveness to methacholine, higher prevalence of skin atopy, elevated serum immunoglobulin E (IgE), and more sputum eosinophilia, compared with their non-asthmatic counterparts. Possible mechanisms by which asthma and bronchiectasis predispose to each other include asthmatic obstruction contributing to development of bronchiectasis, and sensitization of airways with increased lability due to microbial colonization of the ectatic bronchial tree.

Aspergillosis, Allergic Bronchopulmonary↗

PubMed1991

Inherited factors in diffuse bronchiectasis in the adult: a prospective study.

To evaluate the prevalence of inherited respiratory ciliary structure and underlying mucus abnormalities in the diffuse bronchiectasis syndrome, we investigated 53 subjects comprising 38 patients with diffuse bronchiectasis confirmed by high-resolution thoracic computed tomography, ten with chronic bronchitis and no diffuse bronchiectasis and five healthy nonsmoking control subjects. The clinical history was determined by means of a standardized questionnaire. Axonemal abnormalities of respiratory cilia were evaluated on bronchial or nasal mucosa samples by transmission electron microscopy (structure) and stroboscopic observation (function). Cystic fibrosis (CF) and Young's syndrome were detected by means of the sweat test and semen analysis when male infertility was suspected. Among the 38 patients with diffuse bronchiectasis, a primary ciliary dyskinesia (PCD) was detected in five (13%) with a high proportion (range: 55-100%) of cilia showing axonemal ultrastructural abnormalities always involving the dynein arms. The prevalence of this inherited condition was higher in North African (36%) than in European patients (4%) (p less than 0.01). After exclusion of the five patients with PCD, the patients with diffuse bronchiectasis showed axonemal ultrastructural abnormalities similar to those with chronic bronchitis. The diagnosis of underlying mucus disorders was based on two types of criterion, i.e. for CF, sweat chloride levels greater than 80 mmol.l-1, or the combination of diagnostic criteria proposed by Stern et al. Respectively, five (three Young's syndrome and two CF) and seven (one Young's syndrome and six CF) cases of inherited mucus disorders were suspected. Our results showed that PCD was highly prevalent among the adult North African patients with diffuse bronchiectasis but relatively rare in the Europeans.

Adult↗

PubMed1994

Bronchiectasis.

Bronchiectasis, a condition characterized by abnormal and permanent dilatation of pulmonary airways, is an old disease, well recognized in the era before widespread immunization for childhood diseases and the development of antibiotics. After that time, interest in this disease waned. The recognition of bronchiectasis in association with congenital diseases awakened interest in the disease. Review of literature relating to the etiologies of bronchiectasis does not substantiate a congenital origin in most; it appears that the majority of cases of bronchiectasis result from prior infections of the lungs, either viral or bacterial, including mycobacterial. The initial insult triggers an inflammatory change in the bronchial wall that persists even after the episode ends. Over time, the bronchial wall is damaged, making it subject to permanent dilatation. Clinical findings of bronchiectasis include chronic cough with sputum production, often purulent, sometimes hemoptoic. Recurrent pleurisy and fever occur. Spirometry in most patients shows airway obstruction, even if the patient has never smoked. Chest radiographs are usually abnormal, with chronic increased markings caused by infiltrates or scarring, atelectasis, and pleural thickening. Microbiological studies may show a variety of organisms, some commensals, others pathogenic. Diagnosis is made based on the clinical picture with a compatible chest radiograph and confirmed with a high-resolution computed tomography (CT) scan. Therapy of bronchiectasis is directed at airway obstruction, including chest physiotherapy and bronchodilators, and at the infections. Few controlled studies to evaluate any therapy have been performed.

Bronchiectasis↗

PubMed1997

Prevalence of symptomatic bronchiectasis in patients with rheumatoid arthritis.

OBJECTIVE: To evaluate the prevalence of symptomatic bronchiectasis in patients with rheumatoid arthritis. METHODS: Cross-sectional retrospective study of 453 rheumatoid arthritis patients. All patients completed a questionnaire designed to detect manifestations of bronchiectasis and had a chest film taken. Computed tomography of the chest was performed in those patients whose chest film was normal. RESULTS: Thirteen patients (2.9%), all female, had symptoms that met Walker's criteria for definite (n = 7) or probable (n = 6) bronchiectasis. Symptom onset was during childhood or adolescence in 69% of cases and antedated the first symptoms of rheumatoid arthritis in all patients but one. Six of the 13 patients (46%) had chest film abnormalities suggestive of bronchiectasis, and three of the remaining seven patients had abnormal computed tomography findings, yielding a total of nine cases of bronchiectasis confirmed by imaging studies among the 13 patients with suggestive symptoms (69%). This proportion rose to 90% when the three patients who failed to come to their computed tomography appointment were excluded. CONCLUSION: Using a methodological approach similar to that previously used by pneumologists, we found a 2.9% prevalence of symptomatic bronchiectasis in a population of hospitalized rheumatoid arthritis patients, which is higher than the 0.03% prevalence previously reported in the population at large.

Adolescent↗