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.

A total of 287 bronchoscopies were performed in 267 patients infected with the human immunodeficiency virus (HIV). Bronchoalveolar lavage (BAL) and transbronchial biopsies (TBB) were sequentially performed in the same lung, in all cases. Specimens were examined after routine laboratory procedures for microbiologic and histologic analysis. Age ranged from 16 to 78 (median 37.2); 228 were men (85.4%) and 39 were women. Dyspnea was the main symptom in 198 cases (69.0%) and interstitial infiltrates (diffuse or localized) were the most common findings at chest radiographs (179 patients, 62.4%). Endoscopic view was normal in 246 patients (85.7%). Mucosal lesions suggestive of Kaposi's sarcoma (KS) were visible in 12 cases (4.2%). BAL detected 97 infectious agents, simultaneous co-infection occurred in 3 cases. TBB identified 165 pathogens (9 of them in association), anticipating a better yield than BAL in the diagnosis of P. carinii (84 versus 51, respectively) and cytomegalovirus (35 v. 0). Biopsies were unique in establishing the appropriate diagnosis of extrabronchial cancer and describing inespecific pneumonitis. Altogether, a definite diagnosis was obtained by bronchoscopic examination (including endoscopic view, BAL and TBB) in 271 of 287 cases (94.4%). Infections were caused by P. carinii in 105 patients (36.6%), Mycobacterium sp in 40 (13.9%), cytomegalovirus in 35 (12.2%), C. neoformans in 13 (4.5%) etc. Malignancies (including KS) were found in 16 patients (5.6%). More than one pathologic process was present in 28 (9.8%). Nonspecific pneumonitis was diagnosed in 56 cases (19.5%) and normal lung in 20 (7.0%). Complications related to bronchoscopy were pneumothorax (8 patients, 2.8%), haemorrhage (8), thoracic pain (2) and distal pneumonia (2). Chest tubes were required in 4 patients, including one fatal case (mortality rate of 0.3%). Combining BAL and TBB results increases the diagnostic yield of bronchoscopy in AIDS, with a low complication rate. If both techniques are performed, a higher number of infectious agents and more multiple causes of pulmonary disease may be identified.

Keywords: Bronchoscopy/methods. Acquired Immunodeficiency Syndrome/complications. Opportunistic infections related to AIDS/diagnosis.

Introduction: The diagnosis of diffuse lung disease is still a challenge for the pediatric intensive care physician. Routine clinical examinations and laboratory tests are frequently negative. The objective of this study was to evaluate the diagnostic potential, the impact on therapy and the rate of complications of open lung biopsy in children with undiagnosed diffuse lung disease, respiratory failure and inappropriate response to initial therapy. Methods: From January 1987 to January 1997, 29 children with diffuse pulmonary disease of unknown etiology, respiratory failure (PaO2 /FiO2 < 300) and no response to previous treatments were considered for open lung biopsy. Newborns, children with known chronic pulmonary disease and children with untreatable shock or coagulopathy were excluded. All biopsies were performed by a thoracic surgeon by a microthoracotomy in the lung shown to be the most affected by X-ray examination. Tissue samples were analyzed in terms of cultures, light microscopy, electron microscopy and immunofluorescence microscopy, according to the pathologist's decision. Results: All biopsies (100%) resulted in at least one histological diagnosis and in 20 patients (68.9%) it was obtained a specific diagnosis. The most frequent histological patterns found were: non-specific interstitial pneumonitis with variable degrees of fibrosis in 18 cases; bronchiolitis in eight cases and pulmonary hypertension in three cases. Regarding the most frequent specific diagnosis, six children were found with cytomegalovirus infection, three with Pneumocystis carinii, three with adenovirus and three with respiratory syncytial virus infection. These data induced a change in therapy in 20 children (68.9%). The most frequent changes in therapy were the use of corticosteroids in 14 children and a review of the antibiotic regimen in six patients. Seven patients (24.1%) presented with complications that were easily resolved. There were 13 deaths, probably due to the critical conditions of these patients, all unrelated to the procedure. Conclusions: Open lung biopsy, though an invasive procedure, should be considered in the evaluation of selected children with undiagnosed diffuse lung disease, respiratory failure and with no satisfactory response to previous therapies.

Introduction: Bronchoscopy is useful in the diagnosis of tuberculosis suspects with a "negative" sputum smear. The objectives of this study were: to assess yield of bronchoalveolar lavage (BAL) and of transbronchial biopsy (TBB) both in HIV-positive patients (group 1) and immunocompetent individuals (group 2), based on simple and most available laboratory techniques in clinical practice. Patients and methods: The authors performed 319 bronchoscopies in 302 patients. BAL and TBB were sequentially performed in the same lung in all cases. Tuberculosis was diagnosed based on the results of acid-fast stains (AFS) from the lavage (ZN), culture for mycobacteria (LJ), and histological examination of biopsies (HE and ZN). Results: Tuberculosis was the final diagnosis in 28 exams of group 1 (n = 214) and in 35 of group 2 (n = 105). There were abnormal endoscopic findings respectively in 3 and 10 patients. In group 1, AFS were positive in 7 cases, culture of BAL in 14, and TBB in 19. In group 2, AFS were positive in 13 cases, culture in 17, and biopsies in 31. TBB results compared to BAL were in concordance for group 1 (p = 0.823) and different for group 2 (p = 0.022). Immediate results (AFS and TBB) compared to later positive results of culture were similar for group 1 (p = 0.066) but not for group 2 (p = 0.001). There were eight episodes of pneumothorax (2.5%) and one death (0.36%). Conclusions: Both BAL and TBB should be performed whenever possible, aiming towards a higher yield of bronchoscopy in the diagnosis of tuberculosis. Both methods complement each other in immunocompetent patients, whereas TBB establishes a faster diagnosis for HIV-infected patients if associated to AFS.

Keywords: Bronchoscopy/methods. Smear-negative tuberculosis/diagnosis. Opportunistic infections related to AIDS/diagnosis.

This brief communication demonstrates the correlation of persistent respiratory symptoms with functional, tomographic, and transbronchial pulmonary biopsy findings in patients with COVID-19 who had a long follow-up period. We report a series of six COVID-19 patients with pulmonary involvement who presented with persistent dyspnea within 4-15 months of discharge. We performed transbronchial biopsies, and the histopathological pattern consistently demonstrated peribronchial remodeling with interstitial pulmonary fibrosis. Therefore, lung biopsy may be useful in the approach of patients with long COVID-19, although the type of procedure, its precise indication, and the moment to perform it are yet to be clarified. (Brazilian Registry of Clinical Trials-ReBEC; identifier: RBR-8j9kqy [http://www.ensaiosclinicos.gov.br])

Keywords: COVID-19; COVID-19/pathology; Pulmonary fibrosis; Respiratory function tests; Biopsy.

Objective: To investigate the clinical application of CT and CT-guided percutaneous transthoracic needle biopsy (CT-PTNB) in patients with indeterminate pulmonary nodules (IPNs). Methods: We retrospectively studied 113 patients with PNs undergoing CT and CT-PTNB. Variables such as gender, age at diagnosis, smoking status, CT findings, and CT-PTNB techniques were analyzed. Data analysis was performed with the Student's t-test for independent samples the chi-square test, and normal approximation test for comparison of two proportions. Results: Of the 113 patients studied, 68 (60.2%) were male and 78 (69%) were smokers. The diameter of malignant lesions ranged from 2.6 cm to 10.0 cm. Most of the IPNs (85%) were located in the peripheral region. The biopsied IPNs were found to be malignant in 88 patients (77.8%) and benign in 25 (22.2%). Adenocarcinoma was the most common malignant tumor, affecting older patients. The IPN diameter was significantly greater in patients with malignant PNs than in those with benign IPNs (p < 0.001). Having regular contour correlated significantly with an IPN being benign (p = 0.022), whereas spiculated IPNs and bosselated IPNs were more often malignant (in 50.7% and 28.7%, respectively). Homogeneous attenuation and necrosis were more common in patients with malignant lesions (51.9% and 26.9%, respectively) Conclusions: In our sample, CT and CT-PTNB were useful in distinguishing between malignant and benign IPNs. Advanced age and smoking were significantly associated with malignancy. Certain CT findings related to IPNs (larger diameter, spiculated borders, homogeneous attenuation, and necrosis) were associated with malignancy.

Keywords: Solitary pulmonary nodule; Tomography; Image-guided biopsy.

Objective: Bone disease is a common comorbidity in patients with cystic fibrosis (CF). We sought to determine risk factors and identify potential biochemical markers for CF-related bone disease (CFBD) in a unique cohort of CF patients with end-stage lung disease undergoing lung transplantation (LTx) evaluation. Methods: All of the CF patients who were evaluated for LTx at our center between November of 1992 and December of 2010 were included in the study. Clinical data and biochemical markers of bone turnover, as well as bone mineral density (BMD) at the lumbar spine and femoral neck, were evaluated. Spearman's rho and multivariate logistic regression analysis were used. Results: A total of 102 adult CF patients were evaluated. The mean age was 28.1 years (95% CI: 26.7-29.5), and the mean body mass index was 17.5 kg/m2 (95% CI: 17.2-18.2). Mean T-scores were −2.3 and −1.9 at the lumbar spine and femoral neck, respectively, being lower in males than in females (−2.7 vs. −2.0 at the lumbar spine and −2.2 vs. −1.7 at the femoral neck). Overall, 52% had a T-score of < −2.5 at either skeletal site. The homozygous Phe508del genotype was found in 57% of patients without osteoporosis and in 60% of those with low BMD. Mean T-scores were not particularly low in patients with severe CFTR mutations. Although the BMI correlated with T-scores at the femoral neck and lumbar spine, serum 25-hydroxyvitamin D and parathyroid hormone levels did not. Conclusions: CFBD is common in CF patients with end-stage lung disease, particularly in males and patients with a low BMI. It appears that CF mutation status does not correlate with CFBD. In addition, it appears that low BMD does not correlate with other risk factors or biochemical parameters. The prevalence of CFBD appears to have recently decreased, most likely reflecting increased efforts at earlier diagnosis and treatment.

Keywords: Lung transplantation; Cystic fibrosis; Bone density; Osteoporosis.

Objective: To investigate the reliability, internal consistency and validity of the Brazilian Portuguese version of the University of California San Diego Shortness of Breath Questionnaire (UCSD SOBQ) in patients with interstitial lung disease (ILD). Methods: Patients with ILD completed the questionnaire at three different time points, one week apart, with the assistance of two independent assessors. Intra- and inter-rater reliability were analysed via the intraclass correlation coefficient (ICC). Internal consistency was assessed with the Cronbach's alpha coefficient. For the validity analysis, associations between variables were assessed with Spearman's or Pearson's correlation coefficient. Results: Thirty patients with ILD (idiopathic pulmonary fibrosis, connective tissue disease-associated pulmonary fibrosis, sarcoidosis, asbestosis or non-specific interstitial pneumonia) were included (15 men; mean age, 59 ± 10 years; DLCO: 46 [33-64] % predicted). UCSD SOBQ scores showed excellent agreement and internal consistency in the intra-rater analysis (ICC: 0.93 [0.85-0.97]; Cronbach alpha: 0.95) and in the inter-rater analysis (ICC: 0.95 [0.89-0.97]; Cronbach alpha: 0.95), as well as correlating significantly with dyspnoea (as assessed by the Medical Research Council scale; r = 0.56); Medical Outcomes Study 36-item Short-Form Health Survey domains bodily pain, general health, vitality and physical functioning (-0.40 = r = -0.74); six-minute walk distance (r = -0.38); and quadriceps muscle strength (r = -0.41). Conclusions: The Brazilian Portuguese version of the UCSD SOBQ is valid, is reliable and has internal consistency in patients with ILD in Brazil.

Keywords: Lung diseases, interstitial; Dyspnea; Surveys and questionnaires.

Objective: To estimate the frequency of interstitial lung disease in a group of patients with progressive systemic sclerosis, and to describe the clinical, functional and radiological characteristics of the patients studied. Methods: Fifty-eight patients diagnosed with progressive systemic sclerosis were submitted to high-resolution computed tomography of the chest, pulmonary function tests and a blood test for anti-Scl 70 antibodies. Comparisons were drawn between patients with interstitial lung disease and those without. Logistic regression with multivariate analysis was used to identify factors predictive of interstitial lung disease. Results: Of the 58 patients evaluated, 51.7% presented interstitial lung disease on high-resolution computerized tomography scans. Dyspnea and cough were the most common symptoms (seen in 65.5% and 39.7%, respectively). Bronchiolectasis and honeycombing were the most common tomographic abnormalities (observed in 83.3% and 80%, respectively). When compared to individuals without interstitial lung disease, patients with the condition had a comparable frequency of pulmonary and extrapulmonary symptoms but presented progressive systemic sclerosis of longer duration, a higher frequency of crackling rales, higher rates of anti-Scl 70 positivity, lower vital capacity and reduced total lung capacity. Only forced vital capacity < 80% was found to be a predictor of interstitial lung disease. Conclusion: Interstitial lung disease was common in this group of patients with progressive systemic sclerosis. No correlation with symptoms was found, although interstitial lung disease was found to correlate with crackling rales and with anti-Scl 70 positivity. Nevertheless, only reduced forced vital capacity was found to be predictive of interstitial lung disease.

Objective: To assess the risk factors for interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) and to evaluate the association of ILD with the use of methotrexate as well as with joint disease activity. Methods: A retrospective, cross-sectional study conducted between March and December 2019 at a tertiary healthcare center, in a follow-up of RA patients who had undergone pulmonary function tests (PFT) and chest computed tomography. We evaluated the tomographic characteristics, such as the presence of ILD and its extension, as well as joint disease activity. Functional measurements, such as forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO), were also assessed. After this, a multivariate logistic regression analysis was applied in order to identify risk factors associated with ILD. Results: We evaluated 1.233 patients, of which 134 were eligible for this study. The majority were female (89.6%), with a mean age of 61 years old and with a positive rheumatoid factor (86.2%). RA-associated ILD (RA-ILD) was detected in 49 patients (36.6%). We found an association of RA-ILD with age == 62 year, male sex, smoking history and fine crackles in lung auscultation and a decreased DLCO. The indicators of being aged = 62 years old and having moderate or high RA disease activity were both independent factors associated with RA-ILD, with an odds ratio of 4.36 and 3.03, respectively. The use of methotrexate was not associated with a higher prevalence of ILD. Conclusion: Age and RA disease activity are important risk factors associated with RA-ILD. Methotrexate was not associated with the development of RA-ILD in the present study.

Keywords: Rheumatoid arthritis; Interstitial lung disease; Rheumatoid arthritis- associated interstitial lung disease.

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.

Objective: To evaluate the efficacy of the joint use of cervical mediastinoscopy and video-assisted thoracoscopy for the sampling of mediastinal lymph nodes in patients with non-small cell lung cancer (NSCLC) and candidates for pulmonary resection. Methods: Sixty-two patients diagnosed with NSCLC were submitted to cervical mediastinoscopy and video-assisted thoracoscopy. The samples obtained (from paratracheal chains, anterior and posterior subcarinal chains, paraesophageal chains and pulmonary ligament) were submitted to frozen section analysis. The following variables were also evaluated: age; gender; weight loss; diagnostic method; tomographic findings; histological type; staging; and location and size of the primary tumor. Results: In 11 patients, mediastinoscopy showed no involvement of the subcarinal chain, whereas such involvement was identified when video-assisted thoracoscopy was used: positive predictive value = 88.89% (95% CI: 51.75-99.72); negative predictive value = 94.34% (95% CI: 84.34-98.82); prevalence = 17.74% (95% CI: 9.2-29.53); sensitivity = 72.73% (95% CI: 39.03-93.98); and specificity = 98.77% (95% CI: 93.31-99.97). In 60% of the patients with involvement of the posterior subcarinal chain, the primary tumor was in the right inferior lobe. (p = 0.029) Conclusions: The joint use of cervical mediastinoscopy and video-assisted thoracoscopy for the evaluation of posterior mediastinal lymph nodes proved to be an efficacious method. When there is no access to posterior chains by means of ultrasound with transbronchial or transesophageal biopsy, which dispenses with general anesthesia, this should be the method of choice for the correct evaluation of mediastinal lymph nodes in patients with NSCLC.

Keywords: Neoplasm staging; Mediastinoscopy; Biopsy; Lymphatic metastasis.

Objective: Assess the relationship between adherence to long-term oxygen therapy (LTOT) with mortality in patients with chronic obstructive pulmonary disease (COPD) and chronic respiratory failure and their clinical features. Methods: Longitudinal retrospective analysis of 254 patients with COPD and chronic respiratory failure from 2008 to 2016. At baseline, we evaluated the diagnosis, spirometry values, arterial blood gas analysis, blood count, pulse oximetry, body composition and health questionnaires (dyspnea, quality of life, anxiety and depression). For referred adherence analysis to LTOT we included 199 patients, divided according to prescription of oxygen: 12h/day (G1), 15h/day (G2) and 24h/day (G3). The cause of death and dates were studied over the five-year period. Results: In five years we identified 124 deaths (62.3%). No significant difference was found in mortality between the adherence groups (p=0.75) nor did we find differences in the clinical parameters evaluated. LTOT prescription was not associated with mortality (p=0.07). In Cox regression analysis, there was no association between mortality and non-adherence to LTOT (HR: 0.75; IC95%: 0.21-2.70). The risk of mortality was increased in G3 compared with G1 (HR: 7.16; IC 95%: 1.44-35.38) and in those with a higher depression score (HR: 1.35; IC: 1.14-1.59). Conclusion: No association was found between LTOT adherence and mortality in patients with COPD and respiratory failure. There were no clinical differences between the adherence groups.

Keywords: Chronic obstructive pulmonary disease; Oxygen therapy; Cooperation and treatment adherence; Morbidity and mortality indicators.

High-resolution computed tomography (HRCT) is the radiological imaging technique best suited to revealing changes in lung structure. Various HRCT findings, taken together, can represent typical patterns. These patterns, in conjunction with the anatomical distribution of findings and with clinical data, can narrow the differential diagnosis of diffuse interstitial lung disease and, in many cases, indicate the correct diagnosis with a high degree of accuracy. The most common HRCT patterns seen in cases of diffuse interstitial lung diseases are the nodular pattern, linear/reticular opacities, cystic lesions, ground-glass opacities and consolidations. This article reviews the correlations between HRCT patterns and pathologic findings, summarizing the most common causes, as well as detailing the methods of investigation employed in order to diagnose the most common types of chronic diffuse lung disease.

Keywords: Lung diseases, interstitial/pathology; Tomography, X-Ray computed; Diagnostic techniques, respiratory system.

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 predictors of health-related quality of life (HRQoL) in patients with interstitial lung disease (ILD). Methods: A cross-sectional study comprising 63 patients, all of whom underwent lung function testing and the six-minute walk test. The following instruments were used: the Medical Outcomes Study 36-item Short-form Survey (SF-36), the Saint George's Respiratory Questionnaire (SGRQ), the Beck Anxiety Inventory, the Beck Depression Inventory, and the Modified Medical Research Council Dyspnea Scale. Principal component analysis was used in order to reduce the dimensionality of the data, thereby identifying the predictor variables, and multiple linear regression analysis was used in order to identify the explanatory variables. Results: Of the 63 patients, 34 were female. The mean age was 60.1 ± 13.3 years, the mean FVC was 64.17 ± 15.54% of predicted, and the mean DLCO was 44.21 ± 14.47% of predicted. All of the patients evaluated had impaired HRQoL, scoring worst for the SF-36 physical functioning and SGRQ activity domains. Of the patients evaluated, 60.3% and 57.1% showed symptoms of anxiety and depression, respectively. The principal component analysis identified one predictor of physical HRQoL and one predictor of mental HRQoL. Depression had a strong influence on the predictor of mental HRQoL, and the degree of dyspnea had a strong influence on both predictors of HRQoL in the patients evaluated. Variables related to lung function, exercise capacity, and anxiety had no impact on these predictors. Conclusions: In our sample of patients with ILD, the degree of dyspnea had a major impact on the physical and mental HRQoL, and depression had an impact on mental HRQoL.

Keywords: Anxiety; Depression; Dyspnea; Lung diseases, interstitial; Quality of life; Respiratory function tests.

Objective: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). Methods: This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). Results: Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud's phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p > 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. Conclusions: In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.

Keywords: Idiopathic interstitial pneumonias; Autoantibodies; Connective tissue diseases; Autoimmunity.

Objective: Cystic fibrosis (CF) is a chronic multisystemic hereditary disease for which a multidisciplinary approach must be taken. The objective of this study was to show the evolution of a group of patients with CF after the implementation of multidisciplinary treatment. Methods: A retrospective study involving 19 patients (6-29 years of age) under clinical follow-up treatment at the University of São Paulo at Ribeirão Preto School of Medicine Hospital das Clínicas, located in the city of Ribeirão Preto, Brazil. The patients were divided into two groups: 6-12(6-12 years of age) and 13+ (> 12 years of age). We collected data regarding body mass index (BMI), Zscore, Shwachman score (SS), number of exacerbations/year, chronic colonization by Pseudomonas aeruginosa, and spirometric measurements (FVC, FEV1, FEV1%, and FEF25-75%). Data were collected at two different time points (before and after the implementation of the multidisciplinary treatment) and were analyzed with the Wilcoxon signed rank test. Results: The median age at the onset of symptoms was 10 months. In the 6-12 group, only BMI and FVC increased significantly. Although the other spirometric values increased, the differences were not significant. In the 13+ group, there were no significant differences between the two time points. There was a borderline significant decrease in SS and less than significant decreases in the spirometric measurements. However, the number of patients with alterations in volumes and flows decreased in both groups. Conclusions: Although our patient sample was small, the lack of changes in the spirometric parameters might reflect clinical and functional stability. In all of the patients evaluated, clinical, functional, and nutritional parameters remained stable throughout the study period. The implementation of a multidisciplinary approach might have contributed to this result.

Keywords: Cystic fibrosis; Spirometry; Body mass index; Physical therapy modalities.