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In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest scientific knowledge on bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure the quality of content, and are linked to a large amount of online information that includes a wealth of references. These guidelines cover aspects ranging from a consensual definition of bronchiectasis to an evaluation of the natural course and prognosis of the disease. The topics of greatest interest and some new areas are addressed, including epidemiology and economic costs of bronchiectasis, pathophysiological aspects, the causes (placing particular emphasis on the relationship with other airway diseases such as chronic obstructive pulmonary disease and asthma), clinical and functional aspects, measurement of quality of life, radiological diagnosis and assessment, diagnostic algorithms, microbiological aspects (including the definition of key concepts, such as bacterial eradication or chronic bronchial infection), and the evaluation of severity and disease prognosis using recently published multidimensional tools., (Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest therapies available for bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure quality of content, and are linked to a large amount of online information that includes a wealth of references. The guidelines are focused on the treatment of bronchiectasis from both a multidisciplinary perspective, including specialty areas and the different healthcare levels involved, and a multidimensional perspective, including a comprehensive overview of the specific aspects of the disease. A series of recommendations have been drawn up, based on an in-depth review of the evidence for treatment of the underlying etiology, the bronchial infection in its different forms of presentation using existing therapies, bronchial inflammation, and airflow obstruction. Nutritional aspects, management of secretions, muscle training, management of complications and comorbidities, infection prophylaxis, patient education, home care, surgery, exacerbations, and patient follow-up are addressed., (Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Bronchiectasis is caused by many diseases. Establishing its etiology is important for clinical and prognostic reasons. The aim of this study was to evaluate the etiology of bronchiectasis in a large patient sample and its possible relationship with demographic, clinical or severity factors, and to analyze differences between idiopathic disease, post-infectious disease, and disease caused by other factors., Methods: Multicenter, cross-sectional study of the SEPAR Spanish Historical Registry (RHEBQ-SEPAR). Adult patients with bronchiectasis followed by pulmonologists were included prospectively. Etiological studies were based on guidelines and standardized diagnostic tests included in the register, which were later included in the SEPAR guidelines on bronchiectasis., Results: A total of 2,047 patients from 36 Spanish hospitals were analyzed. Mean age was 64.9years and 54.9% were women. Etiology was identified in 75.8% of cases (post-Infection: 30%; cystic fibrosis: 12.5%; immunodeficiencies: 9.4%; COPD: 7.8%; asthma: 5.4%; ciliary dyskinesia: 2.9%, and systemic diseases: 1.4%). The different etiologies presented different demographic, clinical, and microbiological factors. Post-infectious bronchiectasis and bronchiectasis caused by COPD and asthma were associated with an increased risk of poorer lung function. Patients with post-infectious bronchiectasis were older and were diagnosed later. Idiopathic bronchiectasis was more common in female non-smokers and was associated with better lung function, a higher body mass index, and a lower rate of Pseudomonas aeruginosa than bronchiectasis of known etiology., Conclusions: The etiology of bronchiectasis was identified in a large proportion of patients included in the RHEBQ-SEPAR registry. Different phenotypes associated with different causes could be identified., (Copyright © 2016 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Bronchoalveolar lavage (BAL) analysis has been proposed as an objective technique for confirming asbestos exposure. However, the reliability and diagnostic yield of this procedure has not been studied in Spain. The aim of this study was to assess the usefulness of the analysis of asbestos bodies (AB) in bronchoalveolar lavage (BAL) for the diagnosis of asbestos-related diseases (ARD)., Methods: BAL samples from 72 patients (66 male, mean age 66 years) undergoing bronchoscopy were analyzed. Lung tissue from 23 of these patients was also analyzed. Asbestos exposure was assessed by anamnesis and a review of the patient's medical records. BAL and lung samples were processed and AB count was determined by light microscopy. The accepted threshold value to diagnose asbestos-related diseases was 1 AB/ml BAL or 1000 AB/gr dry tissue., Results: Thirty-nine patients reported exposure to asbestos. Of these, 13 (33%) presented AB values above 1 AB/ml BAL. In the 33 non-exposed patients, 5 (15%) presented AB values above 1 AB/ml BAL. There was a significant difference between the AB levels of exposed and non-exposed patients (P=.006). The ROC curve showed that a value of 0.5 AB/ml BAL achieved the most satisfactory sensitivity, 46%, and a specificity of 83%. The correlation between AB levels in BAL and lung was 0.633 (P=.002)., Conclusions: BAL study provides objective evidence of exposure to asbestos. The good correlation between the AB counts in BAL and lung tissue indicates that both techniques are valid for the analysis of asbestos content., (Copyright © 2016 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.)

Pseudomonas aeruginosa is the main pathogen in bronchopulmonary infections in cystic fibrosis (CF) patients. It can only be eradicated at early infection stages while reduction of its bacterial load is the therapeutic goal during chronic infection or exacerbations. Neonatal screening and pharmacokinetic/pharmacodynamic knowledge has modified the management of CF-patients. A culture based microbiological follow-up should be performed in patients with no infection with P.aeruginosa. At initial infection, inhaled colistin (0,5-2MU/tid), tobramycin (300mg/bid) or aztreonam (75mg/tid) with or without oral ciprofloxacin (15-20mg/kg/bid, 2-3weeks) are recommended. In chronic infections, treatment is based on continuous administration of colistin or with a 28-day on-off regimen with tobramycin or aztreonam. During mild-moderate exacerbations oral ciprofloxacin (2-3weeks) can be administered while serious exacerbations must be treated with intravenous combination therapy (beta-lactam with an aminoglycoside or a fluoroquinolone). Future studies will support antibiotic rotation and/or new combination therapies. Epidemiological measures are also recommended to avoid new P.aeruginosa infections and "patient-to-patient transmission" of this pathogen., (Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.)

Background: Bronchiectasis can result from many diseases, which makes the aetiological investigation a complex process demanding special resources and experience. The aetiological diagnosis has been proven to be useful for the therapeutic approach., Objective: Evaluate how accurately and extensive the clinical and aetiological research was for adult bronchiectasis patients in pulmonology outpatient service which were not following a pre-existing protocol., Methods: We retrospectively reviewed the records of 202 adult patients with bronchiectasis, including the examinations performed to explain the aetiology., Results: The mean age of the patients was 54 ± 15 years, there was a predominance of female (63.9%) and non-smoker (70%) patients. Functional evaluation showed a mild airway obstruction. The sputum microbiological examination was available for 168 patients (43.1% had 3 or more sputum examinations during one year). Immunoglobulins and α1-antitrypsin were measured in around 50% of the patients. The sweat test and the CF genotyping test were performed in 18% and 17% of the patients, respectively. The most commonly identified cause was post-infectious (30.3%), mostly tuberculosis (27.2%). No definitive aetiological diagnosis was established in 57.4% of the patients. We achieved a lower aetiological diagnosis if we compare our series with studies in which a diagnostic algorithm was applied prospectively., Conclusions: The general characteristics of our patients were similar with other series. Detailed investigation of bronchiectasis is not a standard practice in our outpatient service. These results suggest that the use of a predefined protocol, based on current guidelines, could improve the assessment of these patients and facilitate the achievement of a definitive aetiology., (Copyright © 2013 Sociedade Portuguesa de Pneumologia. Published by Elsevier España, S.L.U. All rights reserved.)

Hard metal lung disease is an unusual disease which can occur in individuals exposed to hard metals. Clinically, the condition resembles hypersensitivity pneumonitis depending mainly on individual susceptibility, which eventually progresses to pulmonary fibrosis. We present two patients with pulmonary fibrosis, who were actually diagnosed after an exhaustive anamnesis and examination of the tissue by scanning microscope to discard hard metals. The evaluation of wedge biopsies by scanning electronic microscope can be very helpful in those cases without a specific diagnosis., (2009. Published by Elsevier Espana.)

Bronchiectasis is the end result of several different diseases that share principles of management. The clinical course usually involves chronic bronchial infection and inflammation, which are associated with progression. The cause of bronchiectasis should always be investigated, particularly when it can be treated. We recommend evaluating etiology, symptoms, bronchial colonization and infection, respiratory function, inflammation, structural damage, nutritional status, and quality of life in order to assess severity and to monitor clinical course. Care should be supervised by specialized units, at least in cases of chronic bronchial infection, recurrent exacerbations, or when there is a cause that is likely to respond to treatment. Improving symptoms and halting progression are the goals of management, which is based on treatment of the underlying cause and of acute or chronic infections and on the drainage of secretions. Complications that arise must also be treated. Antibiotic prescription is guided by how well infection is being controlled, and this is indicated by the color of sputum and a reduction in the number of exacerbations. We recommend inhaled antibiotics in cases of chronic bronchial infection that does not respond to oral antibiotics, when these cause side effects, or when the cause is Pseudomonas species or other bacteria resistant to oral antibiotics. Inhaled administration is also advisable to treat initial colonization by Pseudomonas species.

Objective: To determine the diagnostic yield achieved with the application of current recommendations for evaluating patients with suspected interstitial lung disease (ILD) and the procedures that must be applied to reach a definitive diagnosis., Patients and Methods: Over a 10-year period, 500 consecutive patients attending an ILD outpatient clinic who showed features of diffuse lung involvement were assessed with a single diagnostic protocol. Results were introduced in a dedicated database and diagnoses for idiopathic interstitial pneumonia were established according to a recent consensus classification., Results: A definitive diagnosis was reached in 427 (85%) patients: in 125 without invasive procedures and in 302 with invasive procedures. In 73 (14.6%) cases a definitive diagnosis was not reached, and patients were placed in the group of unclassifiable interstitial pneumonia. Idiopathic interstitial pneumonia was the predominant group with 193 (39%) patients. The main specific entities included sarcoidosis with 93 (19%) patients, usual interstitial pneumonia with 84 (17%) patients, and hypersensitivity pneumonitis with 75 (15%) patients. Thirty (6%) patients were diagnosed with an illness other than ILD (false ILD). In 332 patients, we performed a total of 433 invasive procedures: transbronchial biopsy in 252 (direct diagnostic yield, 38%, or if used also to exclude other specific diagnosis, 50%), bronchoalveolar lavage in 260 (yield, 5%), and open lung biopsy in 141 (yield, 93%). Hence, following the current diagnostic approach, a definitive diagnosis was established for 85% of patients, for 25% solely on clinical grounds and imaging criteria and for 60% on the basis of invasive procedures. Diagnosis by open lung biopsy was still required for 141 (28%) patients., Conclusions: The diagnostic yield was high when the recommended study protocol was followed. A quarter of the diagnoses were reached with clinical criteria alone, but another quarter could only be made after open lung biopsy.

Background: Our goal was to establish the clinical and genetic characteristics of patients diagnosed with adult-onset cystic fibrosis (CF)., Patients and Method: This was a retrospective observational descriptive comparative study of CF patients according to their age at the time of diagnosis. All adult patients (> 16 years old) attended in our CF Unit until November 2001 were included in the study. Those patients diagnosed of CF at their childhood (< 16 years old) were categorized as Group A patients, and those diagnosed in adulthood (>= 16 years old) were categorized as group B patients. Anthropometric parameters, respiratory and digestive clinical abnormalities, chest and abdominal radiological exams, sputum bacteriology, respiratory function and genetic tests were evaluated. Statistical analysis between groups was performed by comparing chi square test for qualitative values and the Student t test for quantitative values., Results: One hundred and eleven patients (60 women, mean age 28, range 16-69 years) out of a total sample of 245 (45.3%) patients attended at the CF unit were enrolled in the study. Group A included 61 patients (32 women; mean age 23) and group B included 50 patients (28 women; mean age 32). The comparative study between both groups showed that patients in group B were older, had a higher weight and less incidence of initial digestive abnormalities, pancreatic insufficiency, malnutrition, hepatic disease, chronic bronchial colonization by Pseudomonas aeruginosa, admissions, lung transplantation and deaths due to CF. On the contrary, these patients had a higher incidence of pancreatitis, allergic bronchopulmonary aspergillosis at diagnosis and better respiratory function test parameters. The sweat test was negative in 4 patients of group B and 1 of group A. The genetic study showed 31 different CF mutations, from which only 10 were observed in group B., Conclusions: CF can also be diagnosed in adult age. Patients diagnosed in adulthood have less digestive abnormalities, better lung function and different genetic mutations. The sweat test can be negative or undetermined. These patients also display a better prognosis.

Background: The study of contacts of patients with tuberculosis is an important preventive measure which helps to identify the risk factors for contagion, to detect new cases of the disease early and to break the epidemiological chain of transmission., Material and Method: Three thousand and seventy-one contacts of 635 patients with tuberculosis were studied in our department over a period of 6 years. With the aid of uni- and multivariate analyses we established the importance of different factors in the appearance of tuberculous disease and infection in contacts., Results: There were 1,341 tuberculin positive contacts (44%). The proportion increased in relation to the degree of the relationship and the bacillary density of the index case. A hundred and seventy-six new cases of tuberculosis were diagnosed among the contacts (5.7%). To study the risk factors for contagion, we analyzed the characteristics of 322 patients with positive sputum smears and their 1,623 contacts; 124 new cases were diagnosed among them (124/176; 70%). The most important factors for contagion in the multivariate analysis were the closer relationship (odds ratio [OR] = 8.32; confidence interval [Cl] 95% = 3.9-17.6), greater TST reaction of contacts (OR = 4.43; Cl 95% = 2.5-7.7), presence of more than 10 bacilli per microscopic field in the sputum samples of the index case (OR = 1.97; Cl 95% = 1.1-3.3), male contacts (OR = 1.86; Cl 95% = 1.2-2.7) and those younger than 15 years (OR = 1.58; Cl 95% = 1.01-2.45). Conversely, the sex of the index case and the history of previous tuberculosis in contacts did not influence the contagion., Conclusions: The study of contacts offers a high yield in the diagnosis of new tuberculosis cases, because the incidence of the disease is much higher than that of the general population. Moreover, it allows the detection of newly infected subjects in whom the application of chemoprophylaxis prevents the development of the disease, and thus the epidemiologic chain of transmission is broken.

Background: Patients with IgA deficiency may be asymptomatic or may present recurrent infections mainly respiratory. A possible explanation for this variety of symptoms may be the association with a deficiency of IgG subclasses., Methods: Twenty-five patients with a symptomatic IgA deficiency with a mean age of 18 +/- 12 years were studied. The quantification of the IgG subclasses was performed by ELISA test with antisubclass specific monoclonal murine antibodies. Serum from 100 healthy adults was collected to determine normal values of the IgG subclasses., Results: Twenty-five (48%) had a deficiency of associated IgG subclasses (6 IgG2 deficiency, 1 IgG3, 3 IgG4 and 2 combined deficiency). The patient with a deficiency of associated IgG subclasses had more recurrent respiratory infections (chi 2, p < 0.03) and pneumonias (chi 2, p < 0.04). This group also had a greater FEV1 alteration (Student-t test, p < 0.04)., Conclusions: Patients with symptomatic IgA deficiency frequently present IgG subclass deficiency and are more likely to have recurrent respiratory infections and greater changes in pulmonary function.

We assessed the efficacy of fibrinogen-thrombin instillation through the fiberoptic bronchoscope to treat massive hemoptysis in patients to whom embolization of bronchial arteries was not available, was contraindicated or had failed. The fibrinogen-thrombin solution used was Tissucol, which in addition to 2% fibrinogen and 4 U/ml of thrombin, also contained factor XIII an aprotinin. The fibrinogen-thrombin solution was instilled with the aid of the Duplojec system and a 70 cm x 2 mm 4-way catheter. In 53 of the 628 fiberoptic bronchoscopies performed during the study, the indication was hemoptysis > or = 150 ml/12 h. Of these, bronchoscopic instillation of fibrinogen-thrombin was indicated in 5 cases because bronchial artery embolization was impossible. The point of bleeding was located by bronchoscopy in all cases and fibrinogen-thrombin instillation controlled hemoptysis immediately and throughout the follow-up period, which ranged 4 to 10 months. Morning expectoration of blood (< 10 ml) was observed in only 1 patient in the 3 days after treatment. The mean time taken for bronchoscopic exploration was 3 minutes (range, 2-7). In all cases fiberoptic bronchoscopy was performed without complications that might have required the procedure to be suspended. We conclude that the local use of fibrinogen-thrombin or fibrin glue instilled through the fiberoptic bronchoscope to the point of bleeding is a simple, fast and cheap way to control massive hemoptysis on a short and long-term basis.(ABSTRACT TRUNCATED AT 250 WORDS)

Background: The indication for treatment with human gammaglobulin in patients with primary hypogammaglobulinemia is well established. Nonetheless, there are no uniform criteria with regard to dose, periodicity and route of administration., Methods: Twenty-seven patients with common variable immunodeficiency (CVI) who received i.m. or i.v. treatment with gammaglobulin were studied, evaluating the secondary effects, stable levels of IgG achieved, control of symptomatology, clinical evolution and the need for adjuvant therapies., Results: Intravenous administration was more effective than intramuscular administration to achieve higher total IgG serum levels (5.2 +/- 1.2 vs 3.5 +/- 1.6 g/l; p = 0.07) in a shorter period of time (2.1 +/- 1.6 months vs 6.3 +/- 2.8 months; p < 0.01) and with new few secondary effects. The dose and periodicity of the treatment was individualized in each patient on the basis of the needs of consumption and the speed of metabolism of the gammaglobulin, with patients with chronic bronchial suppuration and diarrhea being those requiring the greatest doses (p < 0.0001) and a short interdosis time interval (19.2 +/- 3.1 vs 23.6 +/- 3.6 days; p = 0.01). Treatment with human gammaglobulin allowed the control of recurrent bacterial infection; however, adjuvant treatment with respiratory physical measures and antibiotics were required in patients with chronic bronchial suppuration to avoid progressive alteration of respiratory function., Conclusions: The administration of human gammaglobulin at adequate doses and frequency is effective to control infection, avoid the development of chronic bronchial disease, alteration of pulmonary function and the appearance of other complications. Intravenous route is safer and produces fewer secondary effects than intramuscular administration with the doses and period of the treatment requiring individualization for each patient.

Background: Quantification of the IgG subclasses (IgGS) requires highly sensitive and specific techniques since their molecular structure is more than 95% homologous. At present, ELISA and RIA are the most appropriate techniques; although the variability of these techniques and the possible ethnic differences in serum levels of IgGS oblige each laboratory to establish its own reference values in a normal population, a condition necessary for defining IgGS deficits., Methods: In the present study the normal serum values of IgGS in 100 healthy individuals were established. Serum quantification of the IgGS was performed by optimization of the indirect ELISA technique using AcMo and OMS reference standards H00-03 for the 4 subclasses, calibrated vs the OMS WHO 67/97 pattern., Results: Normal values observed for IgG1 were 2.61-10.81 g/l; IgG2, 1.12-4.08 g/l; IgG3, 0.22-2.88 g/l; and IgG4, 0.05-1.56 g/l obtained as of the mean +/- 2 SD for IgG1 and as of the extent of the values of the remaining 3 subclasses by following a non-normal distribution. The sensitivity of the technique was 0.05 g/l with mean intra and inter analysis coefficients of 3.5% and 10.7% respectively. The correlation between total IgG and the sum of the 4 subclasses showed a r = 0.894 (p less than 0.01)., Conclusions: The ELISA technique using AcMo and OMS reference standards is an adequate method for the quantification of the IgG subclasses. However, given the variability of this technique and in order to avoid error, strict working conditions, such as the use of control sera at 2 concentration levels, should be observed for the 4 IgGS in each of the analytic series and each series should also be duplicated.

We report a microepidemic of tuberculosis (TBC) in a family of 12 members, 4 of which were parenteral drug abusers and 3 had anti-human immunodeficiency virus (HIV) antibodies. Four new cases were simultaneously diagnosed in the investigation of the contacts of a patient with extrapulmonary tuberculosis and acquired immunodeficiency syndrome. We review the requirements for the development of these epidemic outbreaks, both in noninfected communities and in the family contacts, where positive anti-HIV antibodies may increase the risk. We emphasize the importance of a systematic study of contacts in these families and the indication of chemoprophylaxis in all those sharing the same household, without age limit.