Objective: To assess the relative frequency of incident cases of interstitial lung diseases (ILDs) in Brazil. Methods: This was a retrospective survey of new cases of ILD in six referral centers between January of 2013 and January of 2020. The diagnosis of ILD followed the criteria suggested by international bodies or was made through multidisciplinary discussion (MDD). The condition was characterized as unclassifiable ILD when there was no specific final diagnosis following MDD or when there was disagreement between clinical, radiological, or histological data. Results: The sample comprised 1,406 patients (mean age = 61 ± 14 years), and 764 (54%) were female. Of the 747 cases exposed to hypersensitivity pneumonitis (HP)-related antigens, 327 (44%) had a final diagnosis of HP. A family history of ILD was reported in 8% of cases. HRCT findings were indicative of fibrosis in 74% of cases, including honeycombing, in 21%. Relevant autoantibodies were detected in 33% of cases. Transbronchial biopsy was performed in 23% of patients, and surgical lung biopsy, in 17%. The final diagnoses were: connective tissue disease-associated ILD (in 27%), HP (in 23%), idiopathic pulmonary fibrosis (in 14%), unclassifiable ILD (in 10%), and sarcoidosis (in 6%). Diagnoses varied significantly among centers (?2 = 312.4; p < 0.001). Conclusions: Our findings show that connective tissue disease-associated ILD is the most common ILD in Brazil, followed by HP. These results highlight the need for close collaboration between pulmonologists and rheumatologists, the importance of detailed questioning of patients in regard with potential exposure to antigens, and the need for public health campaigns to stress the importance of avoiding such exposure.

Keywords: Lung diseases, interstitial/epidemiology; Alveolitis, extrinsic allergic/epidemiology; Connective tissue diseases/epidemiology, Sarcoidosis/epidemiology; Idiopathic pulmonary fibrosis/epidemiology

Interstitial lung diseases (ILDs) in children constitute a heterogeneous group of rare diseases that have been described and classified according to experiences and research in adults. However, pediatric pulmonologists have observed that the clinical spectrum is broader in children than in adults, and that many of these disorders have different courses and treatment responses. In addition, probably due to the various stages of lung development and maturation, new clinical forms have been described, particularly in infants. This has broadened the classification of ILDs in this age bracket. The understanding that neither the usual definition nor the standard classification of these disorders entirely apply to children has prompted multicenter studies designed to increase knowledge of these disorders, as well as to standardize diagnostic and therapeutic strategies. We have reviewed the conceptualization of ILDs in children, taking into consideration the particularities of this group of patients when using the criteria for the classification of these diseases in adults. We have also made a historical review of several multicenter studies in order to further understanding of the problem. We have emphasized the differences in the clinical presentation, in an attempt to highlight knowledge of newly described entities in young children. We underscore the need to standardize management of laboratory and radiological routines, as well as of lung biopsy processing, taking such knowledge into account. It is important to bear in mind that, among the recently described disorders, genetic surfactant dysfunction, which is often classified as an idiopathic disease in adults, should be included in the differential diagnosis of ILDs.

Keywords: Lung diseases, interstitial; Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/therapy; Child.

Many conditions result in chronic interstitial lung disease (ILD), being classified as fibrosing ILDs, including idiopathic pulmonary fibrosis, connective tissue diseases, sarcoidosis, and fibrotic hypersensitivity pneumonitis. HRCT plays an important role in the clinical evaluation of fibrosing ILDs. Current treatment perspectives are encouraging and reinforce the need for HRCT scans of adequate technical quality for early detection of fibrosing ILD. Despite efforts in this regard, the significance and management of imaging findings of early interstitial lung abnormalities have yet to be clarified. After identification of CT findings consistent with fibrosing ILD, radiologists must be able to identify characteristic morphological patterns and, in some cases, features of specific clinical entities. In cases in which HRCT features are not sufficiently specific for a definitive diagnosis, HRCT can aid in selecting the best site for surgical lung biopsy. CT follow-up is useful for identifying progressive fibrosing ILDs and detecting complications unrelated to the underlying disease, including infections, acute exacerbations, and neoplasms. Automated quantification tools have clinical applicability and are likely to be available for use in imaging analysis in the near future. In addition, incorporation of CT evaluation into scoring systems based on clinical and functional parameters for staging fibrosing disease is likely to become valuable in determining prognosis. Knowledge of the clinical applications of CT evaluation is essential for specialists managing patients with fibrosing ILD and can have a positive impact on the clinical course of the disease.

Keywords: Tomography, X-ray computed; Diagnostic imaging; Pulmonary fibrosis.

Objective: To determine the interobserver and intraobserver agreement in the diagnosis of interstitial lung diseases (ILDs) based on HRCT scans and the impact of observer expertise, clinical data and confidence level on such agreement. Methods: Two thoracic radiologists and two general radiologists independently reviewed the HRCT images of 58 patients with ILDs on two distinct occasions: prior to and after the clinical anamnesis. The radiologists selected up to three diagnostic hypotheses for each patient and defined the confidence level for these hypotheses. One of the thoracic and one of the general radiologists re-evaluated the same images up to three months after the first readings. In the coefficient analyses, the kappa statistic was used. Results: The thoracic and general radiologists, respectively, agreed on at least one diagnosis for each patient in 91.4% and 82.8% of the patients. The thoracic radiologists agreed on the most likely diagnosis in 48.3% (κ = 0.42) and 62.1% (κ = 0.58) of the cases, respectively, prior to and after the clinical anamnesis; likewise, the general radiologists agreed on the most likely diagnosis in 37.9% (κ = 0.32) and 36.2% (κ = 0.30) of the cases. For the thoracic radiologist, the intraobserver agreement on the most likely diagnosis was 0.73 and 0.63 prior to and after the clinical anamnesis, respectively. That for the general radiologist was 0.38 and 0.42.The thoracic radiologists presented almost perfect agreement for the diagnostic hypotheses defined with the high confidence level. Conclusions: Interobserver and intraobserver agreement in the diagnosis of ILDs based on HRCT scans ranged from fair to almost perfect and was influenced by radiologist expertise, clinical history and confidence level.

Keywords: Lung diseases, interstitial; Tomography, X-ray computed; Observer variation.

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.

Keywords: Lung diseases, interstitial; Guidelines as topic; Brazil.

Objective: The incidence of lung disease caused by nontuberculous mycobacteria (NTM) has been increasing worldwide. In Brazil, there are few studies about nontuberculous mycobacterial lung disease (NTMLD), and its prevalence is yet to be known. Our objective was to determine the specific etiology of the disease in the state of Rio Grande do Sul, Brazil, as well as the frequency and diversity of NTM species in our sample of patients. Methods: This is a retrospective analysis of the medical records of patients diagnosed with NTMLD treated in a referral center located in the city of Porto Alegre, Brazil, between 2003 and 2013. Results: Our sample comprised 100 patients. The most prevalent NTM species were Mycobacterium avium complex (MAC), in 35% of the cases; M. kansasii, in 17%; and M. abscessus, in 12%. A total of 85 patients had received previous treatment for tuberculosis. Associated conditions included structural abnormalities in the lungs, such as bronchiectasis, in 23% of the cases; COPD, in 17%; and immunosuppressive conditions, such as AIDS, in 24%. Conclusions: MAC and M. kansasii were the most prevalent species involved in NTMLD in the state, similarly to what occurs in other regions of Brazil. Data on regional epidemiology of NTMLD, its specific etiology, and associated conditions are essential to establish appropriate treatment, since each species requires specific regimens. Most patients with NTMLD had received previous tuberculosis treatment, which might lead to development of resistance and late diagnosis.

Keywords: Nontuberculous mycobacteria; Mycobacterium infections, nontuberculous; Lung diseases.

Objective: To determine the relative frequency of hypomagnesemia among patients with chronic airflow limitation treated as outpatients at a referral center in the northern part of the state of Paraná between 2000 and 2001, as well as to determine whether hypomagnesemia correlates with hypoxia, with other electrolyte disturbances and with the severity of airflow limitation. Methods: This was a descriptive study of the relative frequency of hypomagnesemia in 72 patients with chronic airflow limitation. All of the patients were submitted to blood tests to determine serum levels of magnesium and other electrolytes, as well as to staging of the underlying disease. Results: The prevalence of hypomagnesemia was 27.8%. The mean age was 65 ± 9.9 years, and there was a predominance of males. The mean forced expiratory volume in one second was 1.31 ± 0.52 L. Most of the patients (68.1%) were found to be in the advanced stages of the disease. Hypomagnesemia was not found to correlate with other electrolyte disturbances, hypoxemia or disease stage. Conclusion: The high frequency of patients in the advanced stages is likely attributable to the fact that the outpatient facility is a referral center for the region. Further studies should be conducted in order to determine the probable causes of this high prevalence of hypomagnesemia.

Keywords: Magnesium/blood; Pulmonary ventilation; Pulmonary disease, chronic obstructive; Respiratory insufficiency

Objective: To translate the King's Brief Interstitial Lung Disease (K-BILD) questionnaire to Portuguese and culturally adapt it for use in Brazil. The K-BILD quantifies the health status of patients with ILD. Methods: The process involved the following steps: authorization from the author of the original (English-language) questionnaire; translation of the questionnaire to Portuguese by three translators, working independently; merging of the translations by a committee of specialists; back-translation of the questionnaire to English; revision and readjustment of the back-translation by the committee of specialists; evaluation by the original author; revision of the back-translation; cognitive debriefing (verification of the clarity and acceptability of the Portuguese-language version in the target population-i.e., patients with ILD); and finalization of the Portuguese-language version. Results: In the cognitive debriefing step, 20 patients with ILD were interviewed. After the interviews, the clarity and acceptability index of each question was ≥ 0.8, which is considered acceptable. Conclusions: The Portuguese-language version of K-BILD appears to be easily administered to and understood by patients with ILD in Brazil. To our knowledge, this is the only instrument in Brazilian Portuguese that is designed to evaluate the impact that ILD has on the various aspects of the lives of those it affects.

Keywords: Lung diseases, interstitial; Pulmonary fibrosis; Surveys and questionnaires.

In this study, we analyzed the number of TB cases in an emergency room (ER) and the susceptibility profile of Mycobacterium tuberculosis strains. Patients were selected from among those treated at the Hospital das Clínicas, in the city of Belo Horizonte, Brazil. Between 2002 and 2005, 240 TB patients were identified. Of those, 117 patients (48.7%) were diagnosed in the ER, 72 (61.5%) presenting positive sputum smear microscopy. Drug susceptibility testing was carried out in 90 strains, of which 80 (89%) were sensitive, 9 (10%) were resistant, and 1 (1%) was multidrug resistant. The incidence of positive smear sputum microscopy and resistant TB strains was high, which calls for the immediate adoption of TB control measures in the ER.

Keywords: Tuberculosis; Disease transmission, infectious; Drug resistance.

Macrolides are drugs that have antimicrobial effects, especially against intracellular pathogens. Various studies have shown that macrolides might also have anti-inflammatory effects. Macrolides inhibit the production of interleukins and can reduce pulmonary neutrophilic inflammation. Clinical trials have demonstrated beneficial effects of macrolides in various chronic lung diseases. The objective of this study was to review recent data in the medical literature on the anti-inflammatory effects of macrolides in childhood lung diseases by searching the Medline (PubMed) database. We used the following search terms: "macrolide and cystic fibrosis"; "macrolide and asthma"; "macrolide and bronchiolitis obliterans"; and "macrolide and acute bronchiolitis". We selected articles published in international scientific journals between 2001 and 2012. Clinical studies and in vitro evidence have confirmed the anti-inflammatory effect of macrolides in respiratory diseases. Some clinical trials have shown the benefits of the administration of macrolides in patients with cystic fibrosis, although the risk of bacterial resistance should be considered in the analysis of those benefits. Such benefits are controversial in other respiratory diseases, and the routine use of macrolides is not recommended. Further controlled clinical trials are required in order to assess the efficacy of macrolides as anti-inflammatory drugs, so that the benefits in the treatment of each specific clinical condition can be better established.

Keywords: Macrolides; Asthma; Cystic Fibrosis; Bronchiolitis.

We report the case of a 56-year-old patient who underwent left single lung transplantation for idiopathic pulmonary fibrosis (IPF). Despite the high level of immunosuppression after the surgery, there was rapid progression to IPF in the native (right) lung as demonstrated by thoracoscopic lung biopsy. After 104 days on mechanical ventilation, the patient underwent right lung transplant and was discharged from the hospital on postoperative day 26.

Keywords: Idiopathic pulmonary fibrosis; Lung transplantation; Case reports [publication type].

Objective: To study the clinical, radiological, and histopathological patterns of transbronchial biopsy (TBB) used in order to confirm the diagnosis in patients with clinical suspicion of interstitial lung disease (ILD) treated at a tertiary-care university hospital. Methods: We reviewed the medical records, radiology reports, and reports of transbronchial biopsies from all patients with suspected ILD who underwent TBB between January of 1999 and December of 2006 at the Hospital das Clínicas de Botucatu, located in the city of Botucatu, Brazil. Results: The study included 56 patients. Of those, 11 (19.6%) had a definitive diagnosis of idiopathic pulmonary fibrosis (IPF), the rate of which was significantly higher in the patients in which ILD was a possible diagnosis in comparison with those in which ILD was the prime suspect (p = 0.011), demonstrating the contribution of TBB to the diagnostic confirmation of these diseases. The histopathological examination of the biopsies revealed that 27.3% of the patients with IPF showed a pattern of organizing pneumonia, which suggests greater disease severity. The most common histological pattern was the indeterminate pattern, reflecting the peripheral characteristic of IPF. However, the fibrosis pattern showed high specificity and high negative predictive value. For CT scan patterns suggestive of IPF, the ROC curve showed that the best relationship between sensitivity and specificity occurred when five radiological alterations were present. Honeycombing was found to be strongly suggestive of IPF (p = 0.01). Conclusions: For ILDs, chest CT should always be performed, and TBB should be used in specific situations, according to the suspicion and distribution of lesions.

Keywords: Lung diseases, interstitial; Diagnosis, differential; Bronchoscopy.

Objective: To analyze hospitalizations for respiratory diseases among children under 15 years of age in an area with high levels of environmental pollution. Methods: A cross-sectional study of hospitalizations due to respiratory diseases of patients residing in the city of Tangará da Serra, located in the state of Mato Grosso (Brazilian Amazon region), from 2000 to 2005. Data on hospital admissions were obtained from the Brazilian Unified Health Care System and from Brazilian Institute of Geography and Statistics population estimates. Results: In 2005, the rate of hospitalization for respiratory diseases among children under 15 years of age in the microregion of Tangará da Serra was 70.1/1,000 children. Between 2000 and 2005, there were 12,777 such admissions, of which 8,142 (63.7%) were for respiratory diseases. During the dry season (May to October), the rate of admissions for respiratory diseases was 10% higher than during the rainy season (November to April). The principal causes of admission included pneumonia (90.7%) and respiratory insufficiency (8.5%). Admissions of children under 5 years of age for pneumonia were 4 times the expected number for the city. Children under 12 months of age were the most frequently hospitalized, with an average increase of 32.4 admissions per 1,000 children per year. Conclusions: Tangará da Serra presented a high number of pediatric admissions for respiratory diseases. Therefore, it is logical to consider it a priority area for investigation and monitoring of the environmental risk factors for such diseases.

Keywords: Respiratory tract diseases; Pneumonia; Hospitalization; Climate; Air pollution/Brazil.