Brazilian Journal of Pulmonology

ISSN (on-line): 1806-3756 | ISSN (printed): 1806-3713

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Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy

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

Alfredo Nicodemos Cruz Santana, Telma Antunes, Juliana Monteiro de Barros, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro de Carvalho, Carmen Silvia Valente Barbas

J Bras Pneumol.2008;34(6):362-366

Abstract PDF PT PDF EN Portuguese Text

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.

 


Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: update

Atualização do tratamento das vasculites associadas a anticorpo anticitoplasma de neutrófilos

Alfredo Nicodemos Cruz Santana, Viktoria Woronik, Ari Stiel Radu Halpern, Carmen S V Barbas

J Bras Pneumol.2011;37(6):809-816

Abstract PDF PT PDF EN Portuguese Text

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 Wegener's 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.

 


Keywords: Anti-neutrophil cytoplasmic antibody-associated vasculitis; Wegener granulomatosis; Antibodies, antineutrophil cytoplasmic.

 


Incidence of fatal venous thromboembolism in antineutrophil cytoplasmic antibody-associated vasculitis

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

Alfredo Nicodemos Cruz Santana, Teresa Yae Takagaki, Carmen Silvia Valente Barbas

J Bras Pneumol.2011;37(3):409-411

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A murine model of elastase- and cigarette smoke-induced emphysema: is it an opportunity to understand CT emphysema in humans?

Modelo murino de enfisema induzido por elastase e fumaça de cigarro: uma oportunidade para entender o enfisema na TC em seres humanos?

Alfredo Nicodemos Cruz Santana1

J Bras Pneumol.2017;43(2):83-84

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Pulmonary and cutaneous nocardiosis in a patient treated with corticosteroids

Nocardiose pulmonar e cutânea em paciente usuário de corticosteróide

Bruno Guedes Baldi, Alfredo Nicodemos Cruz Santana, Teresa Yae Takagaki

J Bras Pneumol.2006;32(6):592-595

Abstract PDF PT PDF EN Portuguese Text

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]

 


Noninvasive positive airway pressure: from critically ill patients to physical exercise in outpatients

Pressão positiva não invasiva nas vias aéreas: de pacientes em estado crítico a exercício físico em pacientes ambulatoriais

Vinicius Zacarias Maldaner da Silva1, Alfredo Nicodemos Cruz Santana2

J Bras Pneumol.2017;43(6):405-406

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Thrombosis in small and medium-sized pulmonary arteries in Wegener's granulomatosis: A confocal laser scanning microscopy study

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

Alfredo Nicodemos Cruz Santana, Alexandre Muxfeldt Ab`Saber, Walcy Rosolio Teodoro, Vera Luiza Capelozzi, Carmen Silvia Valente Barbas

J Bras Pneumol.2010;36(6):724-730

Abstract PDF PT PDF EN Portuguese Text

Objective: Wegener's 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.

 


Keywords: Vasculitis; Antibodies, antineutrophil cytoplasmic; Wegener granulomatosis; Thrombosis; Lung; Microscopy, confocal.

 


A very rare cause of dyspnea with a unique presentation on a computed tomography scan of the chest: macrophage activation syndrome

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

Rodrigo Antônio Brandão-Neto, Alfredo Nicodemos Cruz Santana, Debora Lucia Seguro Danilovic, Fabíola Del Carlo Bernardi, Carmen Silvia Valente Barbas, Berenice Bilharinho de Mendonça

J Bras Pneumol.2008;34(2):118-120

Abstract PDF PT PDF EN Portuguese Text

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.

 


Keywords: Macrophage activation; Lymphohistiocytosis, hemophagocytic; Tomography, X-ray computed; Lung diseases, interstitial.

 


 

 


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