Situações especiais nas vasculites pulmonares / Special situations in pulmonary vasculitis

A extensa rede vascular pulmonar e brônquica, associada à grande quantidade de células imunocompetentes, torna os pulmões importante alvo de vasculites imunomediadas. O envolvimento destes vasos sanguíneos pode provocar qualquer sintoma respiratório, dependendo do tipo, local e extensão da lesão no sistema vascular respiratório. Algumas situações ameaçadoras da vida podem resultar, abrindo ou acompanhando o quadro destas patologias e, se não prontamente identificadas e tratadas, podem resultar em risco elevado de morbimortalidade. Destacamos duas destas condições especiais - estenoses de grande vias aéreas e hemorragia alveolar disusa - e descrevemos seus aspectos fisiopatológicos, clínicos e terapêuticos

The extensive pulmonary and bronchial vascular system, associated to large amount of immunocompetent cells, makes the respiratory system an important target of immune mediated vasculitis. These blood vessels involvement can cause any respiratory symptoms, depending on the type, location, and degree of lesion in the pulmonary vascular system. Some life-thereatening conditions may ensue, opening or following the course of those deseases and, if not identified and treated promptly, result in increased risk of morbitidy and mortality. We highlight two of those special situations: large airway stenosis and diffuse alveolar hemorrhage, describing their pathophysiological clinical and therapeutic aspects

Vasculites pulmonares para o pneumologista: uma "cartilha introdutória" / Pulmonary vasculitis for pulmonologists: a 'introductory primer

Vasculites pulmonares são doenças raras, mas com grande possibilidade de produzir morbimortalidade nos indivíduos acometidos, não somente pela sua potencial gravidade multisistêmica, mas também por seu diagnóstico geralmente tardio. A identificação de padrões de doença que remetem a estes diagnósticos e a utilização de novos meios de investigação - incluindo ANCA (anticorpo antineutrofílico citoplasmático), reconhecendo suas vantagens e limitações - são importantes, se quisermos utilizar o moderno arsenal terapêutico da forma adequada e com sucesso. Atenção às resultantes clínicas é fundamental no acompanhamento de longo curso destes pacientes

Pulmonary vasculitis are rare illnesses, although possessing substantial morbi-mortality in affected individuals, not only due to its multisystemic nature, but also because of commonly delayed diagnosis. Disease pattern recognition that remind us of that group of diseases, and the use of modern investigation methods - including ANCA (anti-neutrophil cytoplasmic antibodies), with the knowledge of its pros and cons, are key features if the state of the art therapeutic modalities are to be properly used with success. Attention to clinical outcomes is fundamental in the long range management of these patients

Clinical Year in Review of Pulmonary Vascular Disease / 결핵및호흡기질환

Pulmonary vascular disease is a category of disorders, including pulmonary hypertension, pulmonary embolism or chronic thromboembolic pulmonary hypertension, pulmonary vasculitis, pulmonary vascular disease secondary to chronic respiratory disease, and pulmonary vascular tumor and malformations. This article reviews the recent advances in this wide spectrum of pulmonary vascular diseases.

Involvement of large pulmonary vessels in Behcet's disease:the clinical imaging features of 13 cases / 中国实用内科杂志

Objective To analyze the clinical characteristics of large pulmonary vessel involvement in Behcet's diseaseMethods The clinical data of Behcet's disease patients with large pulmonary vessel involvement,who were treated in Peking Union Medical College Hospital from 1983 to 2008,were retrospectively analyzed.Results Thirteen patients had large pulmonary vessel involvement among a total of 418 patients with Behcet's disease.The 13 patients had a mean age of (37.5?12)years old,with 10(69.2%)having hemoptysis,8(61.5%)having dyspnea.Helical CT/CTPA showed that pulmonary vascular involvement included pulmonary arterial aneurysms(53.8%),pulmonary artery occlusion(46.2%),and pulmonary thrombi(46.2%).All the 13 patients were treated with steroid and immunosuppressant;five received steroid pulse therapy;and four received vascular intervention therapy.The mean follow-up was (3?1.6)years,with a follow-up rate of 92.5%.The 3-year survival rate was 83.9% for the 13 patients.Conclusion The manifestation of pulmonary vascular involvement in Behcet's disease is various,and the diagnosis mainly depends on the manifestations of CT pulmonary angiography.

A Case of Pulmonary Infarction Probably Related with Henoch-Schönlein Purpura

We report a case of pulmonary infarction probably related with Henoch-Schönlein purpura, which presented with purpura on both lower extremities in a 27-year-old woman. The purpura had developed 4 days previously and it had no itching or tend'erness. She complained of mild fatigue, chest tightness, but no dyspnea or hemoptysis. On routine examination, chest radiography showed a poorly defined nodular opacity on the left lung field, and it was diagnosed by a follow up chest CT as a pulmonary infarction secondary to pulmonary vasculitis.

Clinical Manifestations of the Lung Involvement in Behet's Syndrome / 결핵

Background: Behet's syndrome is a chronic multisystemic disease affecting many organs such as skin, mucosa, eye, joint, central nervous system and blood vessels. Lung involvement occurs in 5% of Behet's syndrome and is thought to be due to the pulmonary vasculitis leading to thromboembolism, aneurysm and arteriobronchial fistula. Pulmonary vasculitis in Behet's syndrome is a unique clinical feature, differing from other vascuitis affecting the lung and is one of the major causes of death. Therefore, we examined the incidence, the clinical features, the radioloic findings and the clinical courses of the lung involvement in Behet's syndrome. Methods: We retrospectively reviewed the medical records and radiologic studies of 10 cases of the lung involvement in Behet's syndrome diagnosed at Yongdong Severance Hospital and Severance Hospital from 1986 to 1995. We analysed the clinical features, the radiological findings, the treatment modalities and the clinical courses. Results: 1) The incidence of the lung involvement in Behet's syndrome was 2%(10/487). The male to female ratio was 8: 2 and the mean age was 34 years. The presenting symptom was hemoptysis in 5 of 10 cases, and massive hemoptysis was noted in 2 cases. Other pulmonary symptoms were cough(6/10), dyspnea(4/10), and chest pain(2/10). Other manifestations were oral ulcers(10/10), genital ulcers(9/10), skin lesions(7/10), and eye lesions(6/10). 2) The laboratory findings were nonspecific. The posteroanterior views of chest radiographies showed multiple infiltrates(6/10), nodular or mass-like opacities(4/10), or normal findings(2/10). The chest CT scans showed multifocal consolidations(6/8), and aneurysms of the pulmonary arteries(4/8). The pulmonary angiographies were performed in 3 cases, and showed pulmonary artery aneurysms in 2 cases. The ventilation-perfusion scans in 2 cases of normal chest x-ray showed multiple mismatched findings. 3) The patients were treated with combination therapy consisting of corticosteroids, cyclophosphamide, and colchicine or anticoagulant agents. Surgical resection was performed in one case with a huge aneurysm. 4) We have followed up nine of ten cases. Three cases are well-being with medical therapy, two cases are severely disabled now and four cases died due to massive hemoptysis, massive pulmonary embolism, or sepsis. Conclusion: Pulmonary vasculitis is a main feature of the lung involvement of Behet's syndrome, causing hemorrhage, aneurysmal formation, and/or thromboemboism. The lung involvement of Behet's syndrome is uncommon but is one of the most serious prognostic factors of the disease. Therefore, an aggressive diagnostic work-up for early detection and proper treatment are recommended to improve the clinical course and the survival.

Clinical Manifestations of the Lung Involvement in Behet's Syndrome / 결핵

Background: Behet's syndrome is a chronic multisystemic disease affecting many organs such as skin, mucosa, eye, joint, central nervous system and blood vessels. Lung involvement occurs in 5% of Behet's syndrome and is thought to be due to the pulmonary vasculitis leading to thromboembolism, aneurysm and arteriobronchial fistula. Pulmonary vasculitis in Behet's syndrome is a unique clinical feature, differing from other vascuitis affecting the lung and is one of the major causes of death. Therefore, we examined the incidence, the clinical features, the radioloic findings and the clinical courses of the lung involvement in Behet's syndrome. Methods: We retrospectively reviewed the medical records and radiologic studies of 10 cases of the lung involvement in Behet's syndrome diagnosed at Yongdong Severance Hospital and Severance Hospital from 1986 to 1995. We analysed the clinical features, the radiological findings, the treatment modalities and the clinical courses. Results: 1) The incidence of the lung involvement in Behet's syndrome was 2%(10/487). The male to female ratio was 8: 2 and the mean age was 34 years. The presenting symptom was hemoptysis in 5 of 10 cases, and massive hemoptysis was noted in 2 cases. Other pulmonary symptoms were cough(6/10), dyspnea(4/10), and chest pain(2/10). Other manifestations were oral ulcers(10/10), genital ulcers(9/10), skin lesions(7/10), and eye lesions(6/10). 2) The laboratory findings were nonspecific. The posteroanterior views of chest radiographies showed multiple infiltrates(6/10), nodular or mass-like opacities(4/10), or normal findings(2/10). The chest CT scans showed multifocal consolidations(6/8), and aneurysms of the pulmonary arteries(4/8). The pulmonary angiographies were performed in 3 cases, and showed pulmonary artery aneurysms in 2 cases. The ventilation-perfusion scans in 2 cases of normal chest x-ray showed multiple mismatched findings. 3) The patients were treated with combination therapy consisting of corticosteroids, cyclophosphamide, and colchicine or anticoagulant agents. Surgical resection was performed in one case with a huge aneurysm. 4) We have followed up nine of ten cases. Three cases are well-being with medical therapy, two cases are severely disabled now and four cases died due to massive hemoptysis, massive pulmonary embolism, or sepsis. Conclusion: Pulmonary vasculitis is a main feature of the lung involvement of Behet's syndrome, causing hemorrhage, aneurysmal formation, and/or thromboemboism. The lung involvement of Behet's syndrome is uncommon but is one of the most serious prognostic factors of the disease. Therefore, an aggressive diagnostic work-up for early detection and proper treatment are recommended to improve the clinical course and the survival.