Alfredo Nicodemos Cruz Santana

Destaques das diretrizes de doenças pulmonares intersticiais da Sociedade Brasileira de Pneumologia e Tisiologia

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.

The role of pneumonia scores in the emergency room in patients infected by 2009 H1N1 infection.

Despite the severity of pneumonia in patients with pandemic influenza A infection (H1N1), no validated risk scores associated with H1N1 pneumonia were tested. In this prospective observational study, we analyzed data of consecutive patients in our emergency room, hospitalized because of pneumonia between July and August 2009 in a public hospital in Brazil. The following pneumonia scoring systems were applied: the SMART-COP rule; the Pneumonia Severity Index; and the CURB-65 rule. Of 105 patients with pneumonia, 53 had H1N1 infection. Among them, only 9.5% that had a low risk according to SMART-COP were admitted to ICU, compared with 36.8% of those with the Pneumonia Severity Index score of 1–2 and 49% of those with CURB-65 score of 0–1. The SMART-COP had an accuracy of 83% to predict ICU admission. The SMART-COP rule presented the best performance to indicate ICU admission in patients with H1N1 pneumonia.

Acometimento pulmonar na doença de Behçet: uma boa experiência com o uso de imunossupressores

OBJECTIVE Behcets syndrome, or Behcets 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.

Atualização do tratamento das vasculites associadas a anticorpo anticitoplasma de neutrófilos

In its various forms, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by a systemic inflammation of the small and medium-sized arteries (especially in the upper and lower respiratory tracts, as well as in the kidneys). The forms of AAV comprise Wegeners granulomatosis (now called granulomatosis with polyangiitis), microscopic polyangiitis, renal AAV, and Churg-Strauss syndrome. In this paper, we discuss the phases of AAV treatment, including the induction phase (with cyclophosphamide or rituximab) and the maintenance phase (with azathioprine, methotrexate, or rituximab). We also discuss how to handle patients who are refractory to cyclophosphamide.

Incidência de tromboembolismo venoso fatal em vasculite associada a anticorpo anticitoplasma de neutrófilos

(ANCA)-associated vasculitis. This report is the was 83% on room air. The abnormal results of the ancillary tests were elevated erythrocyte sedimentation rate and positivity for cytoplasmic-ANCA, together with pulmonary embolism (PE), pulmonary consolidation consistent with PE-related pulmonary infarction, and deep venous thrombosis (DVT), as shown in Figure 1b. Consequently, the patient received a diagnosis of active ANCA-associated vasculitis with VTE (without DAH). She was started on enoxaparin (1 mg/kg twice a day), prednisone (60 mg/day), oral cyclophosphamide, sulfamethoxazole, and oxygen (2 L/min, nasal catheter). There was subsequent clinical improvement. However, two days later, the patient died suddenly, and the circumstances of her death were consistent with the occurrence of another PE. The autopsy revealed DVT/PE without DAH or myocardial infarction.The most important message of this report is that pulmonologists should be alert to the possibility of VTE in ANCA-associated vasculitis. Early clinical suspicion of VTE will avoid delays in diagnosis and treatment, thereby promoting better clinical results. Classically, when hemoptysis does occur (in ANCA-associated vasculitis), “it is usually related to nodules or localized infiltrates, rather than DAH”, which does not include PE as a cause of hemoptysis in ANCA-associated vasculitis.

Trombose em artérias pulmonares pequenas e médias em granulomatose de Wegener: um estudo com microscopia confocal por varredura a laser

OBJECTIVE Wegeners granulomatosis (WG) can cause endothelial cell damage and thromboembolic events. Nevertheless, there have been few studies on the pulmonary microcirculation--small and medium-sized pulmonary arteries (SMSPA)--in patients with WG. The objective of this study was to quantify fibrin thrombi in the SMSPA of patients with WG. METHODS We analyzed 24 SMSPA samples collected from six patients with WG and 16 SMSPA samples collected from four patients without WG. In all samples, we used the endothelial cell marker CD34 and confocal laser scanning microscopy in order to detect intravascular fibrin thrombi. We calculated the total vessel area, the free lumen area, and the thrombotic area. RESULTS The mean total vessel area was similar in the WG and control groups (32,604 µm² vs. 32,970 µm², p = 0.8793). Thrombi were present in 22 (91.67%) of the 24 WG group samples and in none of the control group samples (p < 0.0001; OR = 297; 95% CI: 13.34-6,612). The mean thrombotic area was greater in the WG group samples than in the control group samples (10,068 µm² vs. 0.000 µm²; p < 0.0001). In contrast, the mean free lumen area was smaller in the WG group samples than in the control group samples (6,116 µm² vs. 24,707 µm²; p < 0.0001). CONCLUSIONS Confocal laser scanning microscopy revealed a significant association between pulmonary microvascular thrombosis and WG. This suggests a possible role of microvascular thrombosis in the pathophysiology of pulmonary WG, evoking the potential benefits of anticoagulation therapy in pulmonary WG. However, further studies are needed in order to confirm our findings, and randomized clinical trials should be conducted in order to test the role of anticoagulation therapy in the treatment of patients with pulmonary WG.

Uma rara causa de dispnéia com apresentação singular na tomografia computadorizada de tórax: síndrome de ativação macrofágica

Macrophage activation syndrome is a rare and potentially life-threatening disease. It occurs due to immune dysregulation manifested as excessive macrophage proliferation, typically causing hepatosplenomegaly, pancytopenia and hepatic dysfunction. Here, we report an unusual case of macrophage activation syndrome presenting as dyspnea, as well as (reported here for the first time) high resolution computed tomography findings of an excavated nodule, diffuse ground glass opacities and consolidations (mimicking severe pneumonia or alveolar hemorrhage). The patient was successfully treated with human immunoglobulin. We recommend that macrophage activation syndrome be considered in the differential diagnosis of respiratory failure. Rapid diagnosis and treatment are essential to achieving favorable outcomes in patients with this syndrome.

Remembering Other Causes of Alveolar Siderophages : Macrophage Activation Syndrome

2008;133;1055 Chest Alfredo N. C. Santana, Ronaldo A. Kairalla and Carlos R. R. Carvalho Syndrome Siderophages : Macrophage Activation Remembering Other Causes of Alveolar http://chestjournal.chestpubs.org/content/133/4/1055.full.html services can be found online on the World Wide Web at: The online version of this article, along with updated information and ISSN:0012-3692 ) http://chestjournal.chestpubs.org/site/misc/reprints.xhtml ( written permission of the copyright holder. this article or PDF may be reproduced or distributed without the prior Dundee Road, Northbrook, IL 60062. All rights reserved. No part of Copyright2008by the American College of Chest Physicians, 3300 Physicians. It has been published monthly since 1935. is the official journal of the American College of Chest Chest

Assessment scores in H1N1 infection

The usefulness of assessment scores in H1N1 patients is still undefined, the authors compare the STSS score used in the article of Adenjii and Cussak and compare with SMART-COP in a sample of 53 patients

Remembering Other Causes of Alveolar Siderophages

2008;133;1055 Chest Alfredo N. C. Santana, Ronaldo A. Kairalla and Carlos R. R. Carvalho Syndrome Siderophages : Macrophage Activation Remembering Other Causes of Alveolar http://chestjournal.chestpubs.org/content/133/4/1055.full.html services can be found online on the World Wide Web at: The online version of this article, along with updated information and ISSN:0012-3692 ) http://chestjournal.chestpubs.org/site/misc/reprints.xhtml ( written permission of the copyright holder. this article or PDF may be reproduced or distributed without the prior Dundee Road, Northbrook, IL 60062. All rights reserved. No part of Copyright2008by the American College of Chest Physicians, 3300 Physicians. It has been published monthly since 1935. is the official journal of the American College of Chest Chest