The hypothesis that exposure to silica might be associated with a wide range of autoimmune diseases including SLE (systemic lupus erythematosus) has been discussed over the last decade, but only few cases of silicosis and SLE were described in the literature. The authors report the case of a male patient in his fifth decade of life, with previous exposure to silica, who worked as a well digger for ten years. The patient's clinical picture started with articular symptoms, sporadic peaks of fever, anemia, positive antinuclear factor, peripheral (1/10) and homogeneous (1/500) standard, and productive cough. Computed tomography of the chest showed a diffuse interstitial process, bilateral nodules, para-aortic and para-tracheal hilar calcifications, compatible with pulmonary and ganglial silicosis. He developed acute respiratory distress syndrome (ARDS) and died.

Keywords: Systemic lupus erythematosus. Silicosis. Autoimmune diseases.

Systemic lupus erythematosus (SLE) may involve the respiratory tract in several ways, such as through pleuritis, pneumonitis, interstitial disease or pulmonary hypertension. In rare cases, SLE patients present a syndrome characterized by dyspnea, chest pain and abnormalities in pulmonary function testing, although there may be no evidence of major parenchymal lung disease on computerized tomography scans. This condition has come to be known as shrinking lung syndrome. We report a case that meets these diagnostic criteria, emphasizing the pathogenesis proposed, as well as the therapeutic options available.

Keywords: Lupus Erythematosus/Systemic, Respiratory System, Shrinking Lung Syndrome

Severe pulmonary hypertension is a debilitating disease with short life expectancy that often affects young people. Pleuropulmonary complications of systemic lupus erythematosus occur in 50-70% of patients. Severe symptomatic pulmonary hypertension in systemic lupus erythematosus is rare and carries a bad prognosis because a fatal outcome can occur within months. The authors describe, for the first time, a patient with systemic lupus erythematosus with severe pulmonary hypertension and secondary antiphospholipid syndrome who responded favorably to oral sildenafil, after unsuccessful use of prednisone, intravenous cyclophosphamide, warfarin and diltiazem.

Keywords: Vasodilator agents/therapeutic use. Lupus erythematosuos systemic. Antiphospholipid antibodies. Phosphodiesterase inhibitors.

Alveolar hemorrhage leading to respiratory failure is uncommon. Various etiologies have been reported, including systemic lupus erythematosus, which generally presents as pulmonary-renal syndrome. It is believed that the pathogenesis of microangiopathy is related to deposits of immune complexes that lead to activation of cellular apoptosis. The authors report two cases of alveolar hemorrhage and respiratory failure, both requiring mechanical ventilation. The two cases had opposite outcomes after pharmacological therapy. The presence of anti-glomerular basement membrane antibodies in one of the cases demonstrates the multiplicity of physiopathological mechanisms that may be involved. This multiplicity of mechanisms provides a possible explanation for the heterogeneous responses to the available treatments.

Keywords: Lupus erythematosus systemic/etiology. Lupus nephritis/etiology. Respiratory insufficiency/complications.

A síndrome POEMS é uma rara doença de plasmócitos. A ocorrência de hipertensão pulmonar como complicação respiratória da síndrome é pouco frequente e pode estar ligada ao aumento de várias citocinas, quimiocinas e fatores de crescimento como parte dos fenômenos inflamatórios que cercam a fisiopatologia da síndrome POEMS. Descrevemos o caso de uma mulher de 54 anos com síndrome POEMS e hipertensão pulmonar, que foi tratada com corticoide como terapia de primeira linha. Tratava-se de uma paciente com clássicos sintomas dessa síndrome: polineuropatia (confirmada por eletroneuromiografia), organomegalia, hipotireoidismo subclínico, gamopatia monoclonal em dosagem urinária e alterações cutâneas. A cateterização cardíaca direita revelou pressão arterial pulmonar média de 48 mmHg, débito cardíaco de 4,1 L/min e resistência vascular pulmonar de 8,05 Woods. O nível sérico de brain natriuretic peptide (BNP) foi de 150 pg/mL. Nenhuma outra doença foi encontrada durante investigação. Prednisona (1 mg/kg por três meses) foi iniciada, com dramática melhora clínica e funcional, além de normalização dos níveis dos hormônios tireoidianos e de proteína em urina por eletroforese. A pressão arterial pulmonar média caiu para 26 mmHg, o débito cardíaco para 3,8 L/min e a resistência vascular pulmonar para 2,89 Woods. O nível sérico de BNP caiu para 8pg/mL. Nossos achados indicam o potencial papel da corticoterapia como primeira linha de tratamento na hipertensão pulmonar associada à síndrome POEMS. Diante da raridade dessa apresentação, um registro multicêntrico deveria ser desenvolvido para permitir a aquisição de mais dados que suportem essa conduta.

Keywords: Síndrome POEMS; Hipertensão pulmonar; Glucocorticoides.

Objective: To investigate the correlation of HRCT findings with pulmonary metabolic activity in the corresponding regions using 18F-FDG PET/CT and inflammatory markers in patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD). Methods: This was a cross-sectional study involving 23 adult patients with SSc-associated ILD without other connective tissue diseases. The study also involved 18F-FDG PET/CT, HRCT, determination of serum chemokine levels, clinical data, and pulmonary function testing. Results: In this cohort of patients with long-term disease (disease duration, 11.8 ± 8.7 years), a nonspecific interstitial pneumonia pattern was found in 19 (82.6%). Honeycombing areas had higher median standardized uptake values (1.95; p = 0.85). Serum levels of soluble tumor necrosis factor receptor 1, soluble tumor necrosis factor receptor 2, C-C motif chemokine ligand 2 (CCL2), and C-X-C motif chemokine ligand 10 were higher in SSc patients than in controls. Serum levels of CCL2-a marker of fibroblast activity-were correlated with pure ground-glass opacity (GGO) areas on HRCT scans (p = 0.007). 18F-FDG PET/CT showed significant metabolic activity for all HRCT patterns. The correlation between serum CCL2 levels and GGO on HRCT scans suggests a central role of fibroblasts in these areas, adding new information towards the understanding of the mechanisms surrounding cellular and molecular elements and their expression on HRCT scans in patients with SSc-associated ILD. Conclusions: 18F-FDG PET/CT appears to be unable to differentiate the intensity of metabolic activity across HRCT patterns in chronic SSc patients. The association between CCL2 and GGO might be related to fibroblast activity in these areas; however, upregulated CCL2 expression in the lung tissue of SSc patients should be investigated in order to gain a better understanding of this association.

Keywords: Tomography; Lung diseases, interstitial; Scleroderma, systemic; Cytokines.

Systemic sclerosis (SS) is an uncommon disease characterized by small blood vessel vasculopathy and increased connective tissue in the skin and in other organs. The pulmonary involvement is common in SS; however, pulmonary hypertension without interstitial fibrosis is rare. The authors present a case of the diffuse form of SS with isolated pulmonary hypertension. The diagnosis of pulmonary hypertension was suggested by physical examination and confirmed by doppler-echocardiography which allowed for the estimation of the pulmonary artery systolic pressure in 80 mmHg. The authors started treatment with nifedipine; however, as the patient presented side effects, it was not possible to increase the dose beyond 30 mg/day. In this dosage, nifedipine did not decrease the pulmonary arterial pressure.

Keywords: Systemic scleroderma. Nifedipine. Pulmonary hypertension. Vasodilatador agents. Doppler echocardiography.

Bronchoalveolar lavage is a safe and simple technique to evaluate lung disease related to exposure to mineral dusts. The aim of this study was to characterize the lymphocyte subsets in bronchoalveolar lavage of patients with silicosis. Bronchoalveolar lavage was carried out in 26 workers with different forms of silicosis: simple form (n = 12), complicated (n = 13) and 1 patient with acute form of the disease. As a control group, 7 healthy individuals were included. Compared to the control group, silicotic patients showed intense pleocytosis constituted mainly by alveolar macrophages with slight lymphocytosis. Lymphocyte subsets present in the bronchoalveolar fluid (BAL) of normal individuals were mature lymphocytes with phenotype CD2+TCRab (87.3%) and only 2.9% were CD2+TCRgd. CD4/CD8 ratio was 1.8 with few (16%) immature double negative T cells subsets (CD4-CD8-). In contrast, silicotic patients showed reduction of the more mature lymphocyte subset CD2+CD4+, CD2+CD8+ and a great increase (47%) of immature (CD4-CD8-) T cell subsets. No increase in the NK (CD56+) cell population was observed. Biochemical analysis of protein contents and determination of the Ig/albumin ratio characterized local immunoglobulin production within the pulmonary microenvironment. Furthermore, lack of increase of plasma cells, as well as the maintenance of the percentage of B lymphocyte population (CD19+) in the BAL of silicotic patients, favors the hypothesis that the cells responsible for Ig production are possibly located in the interstitial space. Altogether the results suggest development of lymphopoiesis and tertiary lymphoid tissue within the pulmonary microenvironment during the clinical course of silicosis.

Keywords: bronchoalveolar lavage, lymphocyte subsets, silicosis, immunophenotyping

Objective: To correlate tomographic findings with pulmonary function findings, as well as to compare chest X-ray findings with high-resolution computed tomography (HRCT) findings, in patients with silicosis. Methods: A cross-sectional study was conducted in 44 non-smoking patients without a history of tuberculosis. Chest X-ray findings were classified according to the International Labour Organization recommendations. Using a semiquantitative system, the following HRCT findings were measured: the full extent of pulmonary involvement; parenchymal opacities; and emphysema. Spirometry and forced oscillation were performed. Pulmonary volumes were evaluated using the helium dilution method, and diffusing capacity of the lung for carbon monoxide was assessed. Results: Of the 44 patients studied, 41 were male. The mean age was 48.4 years. There were 4 patients who were classified as category 0 based on X-ray findings and as category 1 based on HRCT findings. Using HRCT scans, we identified progressive massive fibrosis in 33 patients, compared with only 23 patients when X-rays were used. Opacity score was found to correlate most closely with airflow, DLCO and compliance. Emphysema score correlated inversely with volume, DLCO and airflow. In this sample of patients presenting a predominance of large opacities (75% of the individuals), the deterioration of pulmonary function was associated with the extent of structural changes. Conclusions: In the early detection of silicosis and the identification of progressive massive fibrosis, HRCT scans are superior to X-rays.

Keywords: Silicosis; Occupational diseases; Tomography, x-ray computed; Respiratory function tests.

Objective: To evaluate the resistance values obtained through the use of the forced oscillation technique in patients with varying degrees of airway obstruction resulting from silicosis. Methods: A total of 40 never-smoking volunteers were analyzed: 10 were healthy subjects with no history of pulmonary disease or silica exposure, and 30 had silicosis. The forced oscillation technique was used to examine the subjects, and spirometry was used as a reference in order to classify the obstruction by degree. This classification resulted in five groups: control (n = 10); normal exam, composed of individuals diagnosed clinically and radiologically with silicosis but presenting normal spirometry results (n = 7); mild obstruction (n = 10); moderate obstruction (n = 8); and severe obstruction (n = 5). Results: The reduction observed in the spirometric values corresponded to a significant increase in the total respiratory resistance (p < 0.001), as well as in airway resistance (p < 0.003). A significant reduction in ventilation homogeneity was also observed (p < 0.004). Conclusion: In individuals with silicosis, the additional respiratory resistance-related data obtained through the use of the forced oscillation technique can complement spirometric data. Therefore, the forced oscillation technique presents great potential for the analysis of such patients.

Keywords: Pneumoconiosis; Silicosis; Forced expiratory volume; Oscillometry; Respiratory function tests

Objective: To evaluate the incidence rates of mycobacterial infections in silicosis patients with systemic autoimmune rheumatic disease (ARD). Methods: This was a retrospective cohort of silicosis patients between January of 1999 and December of 2023. We compared the incidence of tuberculosis and nontuberculous mycobacterial disease (NTM) in patients with silicosis with and without ARD. We also compared the tuberculosis incidence in the overall cohort with general Brazilian population estimates. Results: The study comprised 369 silicosis patients, of whom 35 (9.5%) had ARD. Having ARD did not affect the cumulative incidence of mycobacterial diseases. The risk of tuberculosis was higher in the cohort when compared with that in the adult Brazilian male population (age-adjusted incidence rate ratio = 20.46; 95% CI 14.89-28.13). Conclusions: In this cohort of patients with silicosis, ARD was not associated with the incidence of mycobacterial diseases.

Keywords: Silicosis; Tuberculosis; Mycobacterium infections, nontuberculous; Arthritis, rheumatoid; Scleroderma, systemic; Lupus erythematosus, systemic.

To evaluate the applicability of the 12-Item Short-Form Health Survey (SF-12) as an instrument to measure health-related quality of life in a sample of patients with progressive systemic sclerosis (PSS) through the analysis of its reproducibility and its correlation with functional and clinical parameters. Methods: A test-retest reproducibility study for the comparative analysis of the intraclass correlation coefficients (ICCs) of the SF-12 and the SF-36. A total of 46 patients diagnosed with PSS were studied, regardless of the presence of respiratory symptoms. Results: The physical component summary 12 (PCS-12) score had an ICC of 0.47 (95%CI: 0.05-0.71; p < 0.02), whereas the mental component summary (MCS-12) score had an ICC of 0.72 (95%CI: 0.49-0.84; p < 0.001). The PCS-36 score had an ICC of 0.88 (95%CI: 0.78-0.93; p < 0.001), and the MCS-36 score also had an ICC of 0.88 (95%CI: 0.78-0.93; p < 0.001). Conclusion: The SF-12 is a reliable instrument for measuring health-related quality of life in patients with PSS, since it has been proven to be reproducible. However, this version of the SF-12 should only be used in clinical research settings.

Keywords: Quality of life; Questionnaires; Statistics; Scleroderma, systemic.

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.

A 79 year-old man sought treatment in the emergency room complaining of persistent fever, chest pain, and general fatigue. A chest X-ray showed a giant infectious bulla (24 cm in diameter) in the left lung. The patient had no history of abnormalities on X-rays, and his latest medical check-up, conducted in the preceding year, had produced no abnormal findings. Diagnostic procedures, including bronchoscopy, revealed lung cancer (large cell carcinoma) in the left lower bronchus. The tumor obstructed the airway. Although there have been various reports of giant bullae, their etiology remains unknown. We suggest that an obstruction, such as that caused by the tumor in this case, can lead to air trapping, resulting in the formation of a bulla. In the case of a giant bulla that rapidly increases in size, lung cancer should be included in the differential diagnosis.

Keywords: Lung neoplasms/complications; Infection; Drainage.

This article is a literature review of the psychological aspects of smoking behavior, highlighting personality characteristics of the smoker as an obstacle to smoking cessation. It describes the relation between smoking behavior and personality, and between smoking and the principal psychiatric disorders. Studies reveal that smokers tend to be more extroverted, anxious, tense, and impulsive, and show more traits of neuroticism and psychoticism than do ex-smokers or nonsmokers. The literature also reveals a strong association between smoking and mental disorders, such as schizophrenia and depression. Understanding the psychological factors associated with tobacco smoking and dependence can further the development and improvement of therapeutic strategies to be used in smoking-cessation programs, as well as of programs aimed at prevention and education.

Keywords: Personality; Mental disorders; Smoking.

Objective: To determine the prevalence of factors associated with difficult-to-control asthma. Methods: Patients with severe asthma were selected from the outpatient asthma clinic of the Ribeirão Preto School of Medicine Hospital das Clínicas. The patients were divided into two groups: controlled severe asthma and difficult-to-control severe asthma. After new attempts to optimize the severe asthma treatment, a questionnaire was applied, and additional tests for factors associated with difficult-to-control asthma, such as environmental and occupational exposure, smoking history, social factors, rhinitis/sinusitis, gastroesophageal reflux disease (GERD), obstructive sleep apnea, congestive heart failure (CHF), pulmonary embolism, cystic fibrosis, vocal cord dysfunction, α-1 antitrypsin deficiency, and Churg-Strauss syndrome, were performed. Results: 77 patients with severe asthma were selected, of which 47 suffered from hard-to-control asthma, being 68.1% female, with mean age of 44.4 years (±14.4), and forced expiratory volume in one second of 54.7% (±18.3). The most factors most often associated with difficult-to-control asthma were noncompliance with treatment (68%), rhinitis/sinusitis (57%), GERD (49%), environmental exposure (34%), occupational exposure (17%), smoking history (10%), obstructive sleep apnea (2%), and CHF (2%). At least one of these factors was identified in every case. Conclusions: Noncompliance with treatment was the factor most often associated with difficult-to-control asthma, underscoring the need to investigate comorbidities in the evaluation of patients with this form of the disease.

Keywords: Asthma; Smoking; Gastroesophageal reflux; Patient compliance; Rhinitis; Sinusitis.

Objective: To assess the prevalence of and factors associated with smoking experimentation among adolescents. Methods: Cross-sectional study, using a specific questionnaire to interview 2,883 students from 7th to 10th grade in schools located in the urban area of the city of Cuiabá, in the state of Mato Grosso, Brazil. Prevalence was estimated, and the principal factors related to cigarette experimentation were analyzed. Subsequently, a hierarchical logistic model was used to describe the chances of cigarette experimentation related to the variables being investigated. Results: The prevalence of cigarette experimentation was 30.2%. In the final model of the analysis, the main variables related to cigarette experimentation were low maternal level of education (OR = 2.44; 95% CI: 1.72-3.47); low socioeconomic level (OR = 1.39; 95% CI: 1.01-1.93); studying in a public school (OR = 1.56; 95% CI: 1.22-2.00); being in 10th grade (OR = 3.45; 95% CI: 2.63-4.54); attending school in the evening (OR = 2.44; 95% CI: 1.85-3.22); having divorced parents (OR = 1.23; 95% CI: 1.02-1.49); having been held back for one school year (OR = 2.17; 95% CI: 1.78-2.70); having friends who smoke (OR = 3.75; 95% CI: 2.99-4.70) having a sibling who smokes (OR = 2.44; 95% CI: 1.82-3.27); and being older (age 17-19 years) (OR = 2.44; 95% CI: 1.39-4.17). Conclusions: A high proportion of adolescents have experimented with smoking. After adjusting for confounding variables, the factors most strongly associated with cigarette experimentation were maternal level of education, age of the adolescent, attending school in the evening, having been held back for one school year and having a sibling who smokes. Preventive measures must be directed at adolescents in schools in order to control smoking.

Keywords: Smoking; Adolescent; Students; Risk factors.

Chylothorax occurs when there is rupture, laceration or obstruction of the thoracic duct, resulting in the release of chyle into the pleural space. Chylothorax can occur in cases of congenital lymphatic malformation, lymphoma, mediastinal tumor and infectious disease, as well as during surgical procedures and after traffic accident-related trauma. It can also be idiopathic. The condition presents clinical signs of dyspnea, hypotension, generalized edema and cyanosis. The diagnosis is usually made through thoracocentesis, and the treatment is conservative. Spontaneous chylothorax is an uncommon form of pleural effusion, and its diagnosis should be hypothesized only after all other causes have been ruled out. Herein, we describe a case of spontaneous chylothorax associated with light physical activity at a fitness center.

Keywords: Chylothorax; Pleural effusion; Motor; Case reports [Publication type]

Objective: To evaluate risk factors associated with asthma symptoms in adolescents in the 13- to 14-year age bracket. Methods: This was a cross-sectional study involving adolescents enrolled in randomly selected public schools in the city of Belo Horizonte, Brazil, and conducted with the use of the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire and its supplementary module for risk factor assessment. The ISAAC questionnaire was completed by the students themselves, whereas the supplementary questionnaire was completed by their parents or legal guardians. Variables showing p ≤ 0.25 in the univariate analysis were included in the multivariate analysis. Stepwise regression with backward elimination was used for variable selection. Results: We evaluated 375 adolescents, 124 (33.1%) of whom had asthma symptoms. The final multivariate analysis model revealed that asthma symptoms were associated with birth weight < 2,500 g (p < 0.001), day care center or nursery attendance (p < 0.002), maternal history of asthma (p < 0.001), contact with animals during the first year of life (p < 0.027), current contact with animals outside the home (dogs, cats, or farm animals; p < 0.005), and more than 20 cigarettes per day smoked by parents or other household members (p < 0.02). Conclusions: Exposure to animals in and outside the home is associated with asthma symptoms, as is environmental tobacco smoke exposure. Families, health professionals, and administrators of health care facilities should take that into account in order to prevent asthma and reduce asthma morbidity.

Keywords: Asthma; Risk factors; Adolescent.