Behçet's disease is a systemic disease with clinical manifestations, whose underlying histopathologic lesion is a non-specific vasculitis. Pulmonary artery aneurysms and, more rarely, pleuropulmonary manifestations may be found. Some reports are available in the literature about the use of immunosuppressive agents in these conditions. The authors describe a case in whom clinical remission and radiologic resolution were observed after corticosteroid and cyclophophamide therapy.

Keywords: Behçet's disease. Pulmonary artery aneurysms.

The authors present a case of a patient with Behçet's disease who complained about sudden dyspnea while performing light physical exercises, about non-productive cough, weakness, and frontal headache; he also presented significant weight loss in the last month while being treated for an alleged tuberculosis. After going through an episode of massive hemophthisis, his clinical history and physical examination were reevaluated and his new diagnosis was concluded to be Behçet's disease. Thus, the authors started treatment by giving immunosuppressive drugs and corticosteroids and, as a consequence, regression of the aneurysm was noted. In this article the authors discuss the clinical, radiological, histopathologic and therapeutic features of this syndrome.

Keywords: Behçet's disease. Pulmonary arterial aneurysms.

Objective: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement. Methods: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts. Results: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 ± 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8%, as was the five-year survival rate. Conclusions: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.

Keywords: Behcet Syndrome; Lung diseases, interstitial; Pulmonary circulation; Hypertension, pulmonary; Pulmonary embolism; Alveolar.

Objective: To evaluate the prevalence of pulmonary function abnormalities and to investigate the factors affecting lung function in patients treated for pulmonary tuberculosis. Methods: A total of 214 consecutive patients (132 men and 82 women; 20-82 years of age), treated for pulmonary tuberculosis and followed at a local dispensary, underwent spirometry and plethysmography at least one year after treatment. Results: Pulmonary impairment was present in 102 (47.7%) of the 214 patients evaluated. The most common functional alteration was obstructive lung disease (seen in 34.6%). Of the 214 patients, 60 (28.0%) showed reduced pulmonary function (FEV1 below the lower limit of normal). Risk factors for reduced pulmonary function were having had culture-positive pulmonary tuberculosis in the past, being over 50 years of age, having recurrent tuberculosis, and having a lower level of education. Conclusions: Nearly half of all tuberculosis patients evolve to impaired pulmonary function. That underscores the need for pulmonary function testing after the end of treatment.

Keywords: Respiratory function tests; Tuberculosis, pulmonary; Spirometry; Lung diseases, obstructive.

Objective: To compare patients with and without previous lung disease, in terms of the spirometry results after they had been treated for pulmonary tuberculosis (PTB) and cured, as well as to analyze risk factors related to functional severity. Methods: This was a cross-sectional, multicenter study conducted at four referral centers in Brazil. Patients were divided into two groups: those with a history of lung disease or smoking (LDS+ group); and those with no such history (LDS− group). Patients underwent spirometry (at least six months after being cured). Sociodemographic and clinical data were collected. Results: A total of 378 patients were included: 174 (46.1%) in the LDS+ group and 204 (53.9%) in the LDS− group. In the sample as a whole, 238 patients (62.7%) had spirometric changes. In the LDS+ group, there was a predominance of obstructive lung disease (in 33.3%), whereas restrictive lung disease predominated in the LDS− group (in 24.7%). Radiological changes were less common in the LDS− group than in the LDS+ group (p < 0.01), as were functional changes (p < 0.05). However, of the 140 (79.1%) LDS− group patients with a normal or minimally altered chest X-ray, 76 (54%) had functional changes (p < 0.01). The risk factors associated with functional severity in the LDS− group were degree of dyspnea (p = 0.03) and moderate or severe radiological changes (p = 0.01). Conclusions: Impaired pulmonary function is common after treatment for PTB, regardless of the history of lung disease or smoking. Spirometry should be suggested for patients who develop moderate/severe dyspnea or relevant radiological changes after treatment for PTB.

Keywords: Tuberculosis, pulmonary; Respiratory function tests; Airway obstruction/complications.

Behçet disease has been associated to pulmonary manifestations and may be lethal if not treated correctly. The authors describe a case of a 53 year-old male with aneurysmatic pulmonary artery dilatation, pulmonary hypertension, and hemoptysis. A review is presented about the diagnosis and the medical and surgical treatment of this disease.

Keywords: Behcet's syndrome. Pulmonary artery. Pulmonary hypertension. Aneurysm. Hemoptysis.

Objective: To evaluate the pattern of pro-inflammatory cytokines, anti-inflammatory cytokines and the acute phase response (APR) as markers of the response to treatment of pulmonary tuberculosis. Methods: Twenty-eight patients with pulmonary tuberculosis were evaluated at three time points: pretreatment (T0), treatment month 3 (T3) and treatment month 6 (T6). Levels of interferon-gamma (IFN-γ), tumor necrosis factor-alpha (TNF-α), interleukine-10 (IL-10) and transforming growth factor-beta (TGF-β) were determined using ELISA in the supernatant of peripheral blood mononuclear cell and monocyte culture. Levels of total protein, albumin, globulins, C-reactive protein (CRP), alpha-1-acid glycoprotein (AAG) and erythrocyte sedimentation rate (ESR) were also determined. All of these parameters were also evaluated, only once, in a group of healthy controls. Results: In relation to controls, patients presented cytokine levels and APR that were higher at T0, lower at T3 and either lower (TNF-α, IL-10, TGF-β, AAG and ESR) or normal (IFN-γ and CRP) at T6. Conclusions: For individuals with negative smear sputum microscopy, CRP, AAG and ESR are potential markers of pulmonary tuberculosis and of the need for treatment; CRP (T0 > T3 > T6 = reference) can also be a marker of treatment response. In the patients, the Th0 profile (IFN-γ, IL-10, TNF-α and TGF-β), inducer of and protector against inflammation, predominated at T0, whereas the Th2 profile (IL-10, TNF-α and TGF-β), protecting against the harmful pro-inflammatory effect of the remaining TNF-α, predominated at T6. The behavior of IFN-γ (T0 > T3 > T6 = controls) suggests its use as a marker of treatment response.

Keywords: Acute-phase proteins; Cytokines; Mycobacterium tuberculosis; Tuberculosis/therapy.

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 prevalence of spirometric abnormalities in patients screened for coronary artery disease (CAD) and the risk factors for lung function impairment. Methods: Patients referred for cardiac CT underwent spirometry and were subsequently divided into two groups, namely normal lung function and abnormal lung function. The prevalence of spirometric abnormalities was calculated for the following subgroups of patients: smokers, patients with metabolic syndrome, elderly patients, and patients with obstructive coronary lesions. All groups and subgroups were compared in terms of the coronary artery calcium score and the Duke CAD severity index. Results: A total of 205 patients completed the study. Of those, 147 (72%) had normal lung function and 58 (28%) had abnormal lung function. The median coronary artery calcium score was 1 for the patients with normal lung function and 36 for those with abnormal lung function (p = 0.01). The mean Duke CAD severity index was 15 for the former and 27 for the latter (p < 0.01). Being a smoker was associated with the highest OR for abnormal lung function, followed by being over 65 years of age and having obstructive coronary lesions. Conclusions: The prevalence of spirometric abnormalities appears to be high in patients undergoing cardiac CT for CAD screening. Smokers, elderly individuals, and patients with CAD are at an increased risk of lung function abnormalities and therefore should undergo spirometry. (ClinicalTrials.gov identifier: NCT01734629 [http://www.clinicaltrials.gov/])

Keywords: Pulmonary disease, chronic obstructive; Spirometry; Coronary disease; Tomography, X-ray computed.

Pulmonary artery dissection is a fatal complication of long-standing pulmonary hypertension, manifesting as acute, stabbing chest pain, progressive dyspnea, cardiogenic shock, or sudden death. Its incidence has been underestimated, and therapeutic options are still scarce. In patients with pulmonary hypertension, new chest pain, acute chest pain, or cardiogenic shock should raise the suspicion of pulmonary artery dissection, which can result in sudden death.

Keywords: Hypertension, pulmonary; Chest pain; Pulmonary artery.

Objective: To evaluate the effect of lung ischemic preconditioning (IPC) on normothermic ischemia/reperfusion (I/R) injury in a rat model, quantifying the production of reactive oxygen species. Methods: Forty-seven male Wistar rats were randomized into four groups: control, sham, I/R and IPC. Control group animals were anesthetized and killed by decapitation, after which pneumonectomy was performed and the left lungs were stored in liquid nitrogen. Sham, IPC and I/R group rats were anesthetized, tracheostomized, ventilated, anticoagulated and submitted to left thoracotomy with dissection of the left pulmonary artery for clamping. Sham group rats underwent dissection of the left pulmonary artery, I/R group rats underwent 30 min of total hilar clamping, and IPC group rats underwent 5-min clamping of the left pulmonary artery followed by 30 min of total hilar clamping. Lungs were reperfused for 90 min and ventilated with the same parameters, with additional positive end-expiratory pressure of 1 cmH2O. Hemodynamic and blood gas values were obtained prior to thoracotomy, prior to total hilar clamping, after 30 min of reperfusion and after 90 min of reperfusion. Lipid peroxidation was determined by measuring levels of thiobarbituric acid reactive substances. Results: There were no significant differences among the groups in terms of the levels of thiobarbituric acid reactive substances. Nor were there any significant differences among the sham, I/R and IPC groups in terms of arterial oxygen tension, arterial carbon dioxide tension or hemodynamic values. Conclusions: In an in situ I/R rat model, 5-min IPC of the left pulmonary artery does not attenuate I/R injury.

Keywords: Ischemia; Reperfusion; Organ preservation; Reactive oxygen species.

Idiopathic pulmonary arterial hypertension (PAH) patients with a positive response to acute vasodilator challenge and a clinical response to calcium channel blockers (CCBs) for at least one year are traditionally designated true responders. Nevertheless, little is known about a sustained response to CCBs over longer periods of time. We evaluated the loss of response to CCBs after long-term treatment in a cohort of idiopathic PAH patients previously classified as being true responders. Our data suggest that idiopathic PAH patients can lose clinical response to CCBs even after one year of clinical stability, reinforcing the need for constant multidimensional reevaluation to assess the need for targeted PAH therapies and to classify these patients correctly.

Keywords: Pulmonary hypertension; Vasodilator agents; Calcium channel blockers.

Objective: To compare the efficacy, safety, and tolerability of azithromycin and amoxicillin in the treatment of patients with infectious exacerbation of chronic obstructive pulmonary disease. Methods: This study was conducted at six medical centers across Brazil and included 109 patients from 33 to 82 years of age. Of those, 102 were randomized to receive either azithromycin (500 mg/day for three days, n = 49) or amoxicillin (500 mg every eight hours for ten days, n = 53). The patients were evaluated at the study outset, on day ten, and at one month. Based on the clinical evaluation of the signs and symptoms present on day ten and at one month, the outcomes were classified as cure, improvement, or treatment failure. The microbiological evaluation was made through the culture of sputum samples that were considered appropriate samples only after leukocyte counts and Gram staining. Secondary efficacy evaluations were made in order to analyze symptoms (cough, dyspnea, and expectoration) and pulmonary function. Results: There were no differences between the groups treated with azithromycin or amoxicillin in terms of the percentages of cases in which the outcomes were classified as cure or improvement: 85% vs. 78% (p = 0.368) on day ten; and 83% vs. 78% (p = 0.571) at one month. Similarly, there were no significant differences between the two groups in the secondary efficacy variables or the incidence of adverse effects. Conclusion: Azithromycin and amoxicillin present similar efficacy and tolerability in the treatment of acute exacerbation of chronic obstructive pulmonary disease.

Keywords: Amoxicillin/therapeutic use; Azithromycin/therapeutic use; Bronchitis, chronic/drug therapy;

Primary pulmonary hypertension is a rare, progressive disease with high mortality rates. The treatment of primary pulmonary hypertension is still based on high-cost drugs, not yet available in developing countries. Recently, the use of sildenafil as an alternative drug for primary pulmonary hypertension treatment has been reported. It is reported a case of a 21 year-old female patient with primary pulmonary hypertension, who presented an acute worsening of symptoms and decrease of oxygen saturation. The investigation revealed the existence of a patent oval foramen not previously seen, with right-to-left shunt. Sildenafil treatment was instituted in escalating doses. After 40 days of treatment, the echocardiogram showed resolution of the shunt, concomitant to oxygenation improvement. The authors believe that sildenafil is a feasible alternative for primary pulmonary hypertension treatment, although larger clinical trials are necessary to determine its clinical safety and efficacy.

Keywords: Pulmonary hypertension/therapy. Vasodilator agents/administration & dosage.