Thoracic manifestations of Behçet syndrome: radiographic and CT findings in nine patients.

Ahn, Joong Mo; Im, Jung-Gi; Ryoo, Jeongmin; Kim, S J; S, Y; Choi, Yo Won; Oh, Y W; Yeon, Kyung Mo; Han, Man-Seok

doi:10.1148/radiology.194.1.7997553

Cited by 64 publications

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“…Parenchymal opacity and, in particular, airspace consolidation observed on chest radiography or CT was previously reported to be an indication of pulmonary infarction in the clinical setting [5,29]. Thus, our observations on thoracic CT scans of animals with photodynamically induced infarction are in accord with clinical findings.…”

Section: Model Of Targeted Pulmonary Infarctionsupporting

confidence: 89%

“…Pulmonary embolism is more frequently associated with lung infarction in lung transplant recipients (37.5%), probably due to the absent or poorly developed collateral bronchial circulation [4]. Other conditions, such as vasculitis, angioinvasive infections, and sickle cell disease, can also lead to pulmonary infarction [5]. Pulmonary infarction is also a frequent complication in immunocompromised patients with pulmonary aspergillosis, often resulting in fatal consequences [6,7].…”

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confidence: 99%

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Laser-targeted photosensitizer-induced lung injury: Noninvasive rat model of pulmonary infarction

Chrastina¹,

Schnitzer²

2011

Experimental Lung Research

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Pulmonary infarction is a life-threatening lung injury that requires rapid and accurate diagnosis for proper treatment. Targetable and reproducible small-animal models that would allow experimental development and preclinical evaluation of diagnostic methods for detecting pulmonary infarction are critically missing. The authors report here a novel procedure to selectively induce pulmonary infarction by photodestructive laser-light irradiation in a targeted location within a specific lung compartment after administration of a photosensitizer. Histopathological analysis of the illuminated lung tissue revealed massive hemorrhage and vascular occlusion after acute injury localized to the site of irradiation. Collapse of alveolar structure, neutrophil influx, and necrosis were subsequently observed. Computed tomography (CT) scans showed evidence of abnormal density and airspace consolidation in the irradiated area of the lung, but not elsewhere in the lung compartment. Perfusion imaging using 99mTc-labeled macroaggregated albumin by single-photon emission computed tomography revealed diminished scintigraphic signal in the opaque area of infarcted lung tissue. The histological changes, CT findings, and perfusion characteristics of pulmonary infarction are mimicked using laser-irradiated, photosensitizer-mediated photodestruction to selectively induce chronic lung injury in a localized area. This small-animal model can be easily and readily used for targeted induction of pulmonary infarction in a designated area of lung compartment and offers the potential for use in evaluating novel diagnostic and therapeutic methods.

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Section: Model Of Targeted Pulmonary Infarctionsupporting

confidence: 89%

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confidence: 99%

Laser-targeted photosensitizer-induced lung injury: Noninvasive rat model of pulmonary infarction

Chrastina¹,

Schnitzer²

2011

Experimental Lung Research

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“…6 Pulmonary artery aneurysms may manifest as hilar enlargement or round, lobulated opacities on chest radiographs. 16 Computed tomography has largely replaced angiography as the diagnostic tool and magnetic resonance imaging can also be useful for diagnosis of PAAs, but appears to be less sensitive than computed tomography for diagnosis of small PAAs. 2 Erkan et al 2 reported that PAAs are most often located in the right lower lobar arteries, followed by bilateral main pulmonary branches.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary artery aneurysms in Behçet disease

Yuan

2014

J. vasc. bras.

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Pulmonary artery aneurysms (PAAs) are the most common type of pulmonary involvement in Behçet' s disease. However, the relationships between clinical features and prognosis have not been sufficiently evaluated. This article describes the results of a comprehensive review, revealing that PAAs have a predilection for hemoptysis manifestations, increased dimensions, right lower lobar location, multiplicity and concurrent intramural thrombus formation. Surgical intervention was needed in one third of patients. Patients with massive hemoptysis and PAA rupture warranted emergency operations. Conservatively treated patients were prone to PAA progression; interventional embolization was associated with higher risks of recurrence and reintervention for PAAs; and surgically treated patients exhibited the highest mortality rates. In conclusion, PAAs in Behçet' s disease are characterized by a predilection for hemoptysis manifestations, right lower lobar location, multiplicity, and concurrent intramural thrombus formation. Both the condition itself and the surgical operations it warrants are linked with high mortality due to PAA hemorrhage.Keywords: hemoptysis; therapeutic embolization; vasculitis. ResumoOs aneurismas das artérias pulmonares (PAA) são as manifestações mais comuns dos pulmões na doença de Behçet. No entanto, as relações entre as características clínicas e o prognóstico ainda não foram devidamente explicadas. O objetivo do presente artigo foi fazer uma ampla revisão da literatura sobre esta questão. As fontes de dados contaram com uma ampla revisão bibliográfica dos anos de 1990 a 2013, sobre os seguintes temas: doença de Behçet, síndrome de HughesStovin, aneurisma de artéria pulmonar e pseudoaneurisma da artéria pulmonar. Os PAA evoluíram com predileção por hemoptise, aumento de dimensões, localização no lobo inferior direito, multiplicidade e formação de trombo intramural. A intervenção cirúrgica foi necessária em um terço dos pacientes. O tratamento cirúrgico emergencial foi indicado na vigência de ruptura do PAA e de hemoptise maciça. Os pacientes tratados conservadoramente evoluíram com propensão para a progressão do PAA. A embolização dos PAA foi associada a uma taxa maior de recidiva e de reintervenção. Houve diferença significativa entre os grupos quanto às taxas de mortalidade, tendo o grupo do tratamento cirúrgico apresentado a maior taxa. Dentre as variáveis citadas, a hemoptise, o envolvimento da artéria lobar e a ruptura do PAA foram fatores preditivos de maior risco de mortalidade. Houve diferenças significativas nas taxas de mortalidade entre os pacientes cirúrgicos e intervencionistas, e entre os três grupos de pacientes: cirúrgico, intervencionista e conservador. Os pacientes tiveram uma sobrevida global de 61,7% em um seguimento médio de 22,5 meses. Os PAA, na doença de Behçet, apresentaram as seguintes predileções: tendência a hemoptise, multiplicidade, localização no lobo inferior direito e presença de trombos intramurais. As rupturas e hemorragias dos PAA, aliadas ao necessário tr...

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“…(6) In addition, small sample size is a problem inherent to studies of rare manifestations of lowprevalence diseases. (4,5,17,18) In summary, patients with Behcet's diseaserelated pulmonary involvement can present good survival with appropriate immunosuppressive therapy. In addition, Behcet's disease must be recalled as a cause of alveolar hemorrhage and pulmonary hypertension.…”

Section: Discussionmentioning

confidence: 99%

“…However, there have been no randomized controlled studies evaluating these options in treatment, and only a few case series have been published on the topic. (3)(4)(5) A recent cumulative analysis suggested good survival in patients receiving immunosuppressive therapy. (6) The aim of this study was to analyze clinical and tomographic evolution, as well as the survival, of patients with Behcet's disease-related pulmonary involvement treated at our facility, all of whom received immunosuppressive therapy.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2008

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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 hemorrhage. ResumoObjetivo: A doença de Behçet (DB) representa uma patologia sistêmica, cuja sobrevida se relaciona com a presença de acometimento pulmonar. Entretanto, sugere-se que pacientes com diferentes tratamentos podem apresentar diferentes prognósticos. O objetivo deste estudo foi avaliar a evolução clínica e tomográfica, bem como a sobrevida deste pacientes com acometimento pulmonar relacionado à DB acompanhados em nosso serviço. Métodos: Uma análise retrospectiva de nossa experiência com pacientes com acometimento pulmonar relaionado a DB acompanhados de 1 de Janeiro de 1988 a 30 de Abril de 2006. Os dados clínicos, radiológicos, terapêuticos e de sobrevida foram obtidos dos prontuários médicos. Resultados: Foram identificados 9 pacientes, com idade média de 34 ± 11,5 anos, sendo 7 deles do sexo masculino. Os achados radiológicos foram aneurisma de artéria pulmonar (AAP) em 8 pacientes, embolia pulmonar em 3 (resultando em uma incidencia de 5,11 casos/100 paciente-anos), hemorragia alveolar em 1 e hipertensão pulmonar em 1 de 9 doentes. O tratamento consistiu-se de prednisona mais clorambucil (ou ciclofosfamida ou micofenolato de mofetil) em todos os...

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Thoracic manifestations of Behçet syndrome: radiographic and CT findings in nine patients.

Abstract: Chest radiographic findings of thoracic Behçet syndrome are variable and nonspecific. CT can be helpful in the assessment of the syndrome by showing thrombosis of the superior vena cava and characteristic aneurysms of the pulmonary arteries.

Cited by 64 publications

References 0 publications

Laser-targeted photosensitizer-induced lung injury: Noninvasive rat model of pulmonary infarction

Laser-targeted photosensitizer-induced lung injury: Noninvasive rat model of pulmonary infarction

Pulmonary artery aneurysms in Behçet disease

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

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