In Brazil, research on pulmonology has become increasingly more visible in recent years. In addition to the Brazilian Journal of Pulmonology, other journals have contributed to that by publishing relevant articles in this area. The objective of this article was to briefly report the most relevant studies on pulmonology that were published in other important Brazilian journals between 2009 and 2010. Altogether, there were 56 articles related to the various subareas that compose the field of respiratory diseases.

Keywords: Pulmonary medicine; Research; Brazil.

Nocardiosis is a localized or disseminated infection caused by gram-positive bacteria of the genus Nocardia. The infection most commonly affects the lungs, skin and central nervous system. Nocardiosis principally occurs in individuals with cellular immunodeficiency and should be considered in the differential diagnosis when such individuals present respiratory, cutaneous or neurological alterations. Herein, we report a case of pulmonary and cutaneous nocardiosis in a patient receiving oral corticosteroids to treat bronchiolitis obliterans accompanied by organizing pneumonia of unknown origin. After long-term treatment with sulfamethoxazole-trimethoprim, the clinical and radiological profile improved.

Keywords: Nocardia infections; Lung diseases; Bronchiolitis obliterans; Adrenal cortex hormones; Case reports [Publication type]

Benign tracheal tumors are rare, recurrent papillomatosis being the most common. They often simulate obstructive pulmonary diseases, such as asthma and chronic obstructive pulmonary disease, and patients with benign tracheal tumors often undergo long-term treatment for such diseases, without any improvement, Therefore, these tumors should be included in the differential diagnosis in patients presenting tracheobronchial tree obstruction. This report describes the case of a patient with a tracheal polyp. The patient presented symptoms for three years, and the spirometry findings suggested intrathoracic obstruction. The patient presented complete clinical and spirometric recovery after bronchoscopic resection of the tumor.

Keywords: Polyps; Airway obstruction; Spirometry; Bronchoscopy.

Objective: To assess blockade of matrix metalloproteinase (MMP)-2 and MMP-9, as well as the variation in FEV1, in patients with lymphangioleiomyomatosis (LAM) treated with doxycycline (a known MMP inhibitor) for 12 months. Methods: An open-label, single-arm, interventional clinical trial in which LAM patients received doxycycline (100 mg/day) for 12 months. Patients underwent full pulmonary function testing, a six-minute walk test, and quality of life assessment, as well as blood and urine sampling for quantification of MMP-2, MMP-9, and VEGF-D levels-at baseline, as well as at 6 and 12 months after the initiation of doxycycline. Results: Thirty-one LAM patients received doxycycline for 12 months. Although there was effective blockade of urinary MMP-9 and serum MMP-2 after treatment, there were no significant differences between pre‑and post-doxycycline serum levels of MMP-9 and VEGF-D. On the basis of their response to doxycycline (as determined by the variation in FEV1), the patients were divided into two groups: the doxycycline-responder (doxy-R) group (n = 13); and the doxycycline-nonresponder (doxy-NR) group (n = 18). The patients with mild spirometric abnormalities responded better to doxycycline. The most common side effects were mild epigastric pain, nausea, and diarrhea. Conclusions: In patients with LAM, doxycycline treatment results in effective MMP blockade, as well as in improved lung function and quality of life in those with less severe disease. However, these benefits do not seem to be related to the MMP blockade, raising the hypothesis that there is a different mechanism of action. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-6g8yz9 [http://www.ensaiosclinicos.gov.br])

Keywords: Lymphangioleiomyomatosis; Doxycycline; Matrix metalloproteinases; Respiratory function tests.

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild or benign symptoms of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.

Keywords: Idiopathic interstitial pneumonias; Autoantibodies; Connective tissue diseases; Autoimmune diseases; Diagnosis, differential.

Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

Keywords: Pulmonary fibrosis; Emphysema; Hypertension, pulmonary; Lung diseases, interstitial.

In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.

Keywords: Lymphangioleiomyomatosis; Spirometry; Goserelin.

Objective: To determine the frequency of near-fatal asthma in a group of severe asthma patients, as well as the clinical characteristics and prognosis of these patients within a one-year follow-up period. Methods: A prospective study involving 731 low-income patients with severe asthma treated at a referral outpatient clinic located in the city of Salvador, Brazil. The patients were submitted to spirometry at admission, received medications for asthma, and were monitored regarding the frequency of asthma exacerbations during the follow-up period. A subsample of 511 patients also completed questionnaires regarding asthma symptoms and asthma-related quality of life. Results: Of the 731 patients studied, 563 (77%) were female. The median age was 47 years, and 12% were illiterate. Most of the patients had rhinitis, and 70 patients (10%) reported near-fatal asthma prior to admission. Of these 70 patients, 41 (59%) reported having been intubated previously. The patients reporting a history of near-fatal asthma at admission were more likely to have asthma exacerbations during the follow-up period and to respond poorly to therapy than were those not reporting such a history. At the end of the follow-up period, the scores on the two questionnaires were similar between the two groups of patients. Conclusions: The frequency of near-fatal asthma was high in this group of low-income patients with severe asthma. The patients with a history of near-fatal asthma had a worse prognosis than did those without such a history, although both groups had received the same kind of treatment. Curiously, the intensity of symptoms and the quality of life at the end of the study were similar between the two groups.

Keywords: Asthma/prevention and control; Asthma/complications; Quality of life; Prognosis.

On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases.

Keywords: Paracoccidioidomycosis; Cysts; Multidetector computed tomography.

Introduction: Surgical procedures requiring aortic occlusion have been associated with pulmonary reperfusion injury. The authors studied the pulmonary edema in a reperfusion injury model by aortic occlusion in rats. Material and methods: Thirty-three Wistar rats were anesthetized with intraperitoneal injection of sodium pentobarbital (20 mg/kg). Using a midline abdominal incision, the infra-renal aorta was isolated and the animals were randomized into three groups. Ischemia-reperfusion group (IRG, n = 5): rats that underwent 30 min of aorta occlusion and were followed during 120 min of reperfusion. Ischemia group (IG, n = 5): rats that underwent 30 min of aorta occlusion. Control group (CG, n = 5): rats that underwent sham operations without aorta occlusion and were followed during 150 min. Histopathologic examination of the right lung was performed. The pulmonary edema was studied by morphometric analysis and so was the leukocytes infiltration. Eighteen rats (6 rats in each group) were studied with respect to lung fresh/dry weight ratio. ANOVA was used to analyze the morphometric results and the lung fresh/dry weight ratio, with Bonferroni adjustment for paired multiple comparisons. Animals of the IRG presented more pulmonary edema than those of the IG and CG (0.24 vs. 0.17 and 0.17, p < 0.001). There was no difference between the groups regarding the lung fresh/dry weight ratio. There were more inflammatory cells in the lungs of the rats submitted to ischemia-reperfusion. The authors concluded that infra-renal abdominal aorta cross-clamping and unclamping are associated with pulmonary edema. This edema is not induced by elevation of the hydrostatic pressure due to the aortic occlusion, since it was not seen in animals that were only submitted to ischemia.

Keywords: ischemia, reperfusion, pulmonary edema, inbred strains rats

The use of immunobiological agents for the treatment of autoimmune diseases is increasing in medical practice. Anti-TNF therapies have been increasingly used in refractory autoimmune diseases, especially rheumatoid arthritis, with promising results. However, the use of such therapies has been associated with an increased risk of developing other autoimmune diseases. In addition, the use of anti-TNF agents can cause pulmonary complications, such as reactivation of mycobacterial and fungal infections, as well as sarcoidosis and other interstitial lung diseases (ILDs). There is evidence of an association between ILD and the use of anti-TNF agents, etanercept and infliximab in particular. Adalimumab is the newest drug in this class, and some authors have suggested that its use might induce or exacerbate preexisting ILDs. In this study, we report the first case of acute ILD secondary to the use of adalimumab in Brazil, in a patient with rheumatoid arthritis and without a history of ILD.

Keywords: Lung diseases, interstitial; Arthritis, rheumatoid; Antirheumatic agents; Antibodies, monoclonal, humanized/adverse effects.

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.

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.