Pulmonary artery aneurysms in Behçet syndrome

Hamuryudan, Vedat; Er, Tarik; Seyahi, Emire; Akman, Canan; Tüzün, Hasan; Fresko, İzzet; Yurdakul, Sebahattin; Numan, Fürüzan; Yazıcı, Hasan

doi:10.1016/j.amjmed.2004.05.027

Cited by 230 publications

(143 citation statements)

References 16 publications

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“…3 A decade later, 5-year survival had increased to 62%. 4 The present study displayed an overall survival of 76.9% at a mean follow-up of 21.6 months.…”

Section: Discussionsupporting

confidence: 50%

“…119 Marked intimal thickening with degenerative changes in the medial layer from the lobar branches to the arterioles can explain hemoptyisis and PAA rupture. 120 Bronchopulmonary fistulas found during autopsies of patients who died of sudden hemoptysis 43 and in surgical specimens resected from patients with massive hemoptysis 10,110 were apparently the causative etiology of life-threatening hemoptysis. Aneurysms may be single or multiple, unilateral or bilateral, saccular or fusiform, and may be located in the main pulmonary artery or lobar or segmental arteries.…”

Section: Discussionmentioning

confidence: 99%

“…11 Focal hemorrhages or infarct areas can present in the lung parenchyma adjacent to the aneurysms. 120 Thrombosed aneurysms cause ischemia and infarction in the pulmonary parenchyma. 6 Pulmonary artery aneurysms may manifest as hilar enlargement or round, lobulated opacities on chest radiographs.…”

Section: Discussionmentioning

confidence: 99%

“…2 Thrombosis usually develops as a consequence of the underlying extensive vasculitis. 3,4 Currently, pulmonary artery aneurysms are the second most common type of arterial involvement in Behçet's disease, preceded by aortic aneurysms. 2 Hemoptysis of varying degrees up to 500 ml was the most common symptom of PAAs, observed in 79%.…”

Section: Introductionmentioning

confidence: 99%

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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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“…3 A decade later, 5-year survival had increased to 62%. 4 The present study displayed an overall survival of 76.9% at a mean follow-up of 21.6 months.…”

Section: Discussionsupporting

confidence: 50%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Pulmonary artery aneurysms in Behçet disease

Yuan

2014

J. vasc. bras.

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“…A regimen including pulses of methylprednisolone followed by a combination of high dose prednisone and CYC was used traditionally in Turkey. 69 Cases of successful treatment with TNF-α inhibitor for patients with BD and pulmonary artery aneurysm have been reported. [70][71][72] There is a case report of a patient with BD, DVT and pulmonary artery aneurysm treated with inferior vena cava (IVC) filter, coil embolization for pulmonary artery aneurysm, anticoagulation and immunosuppression.…”

Section: Behçet's Diseasementioning

confidence: 99%

Venous thromboembolism in systemic autoimmune diseases: A narrative review with emphasis on primary systemic vasculitides

Tamaki

Khasnis

2015

Vasc Med

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Venous thromboembolism (VTE) is a prevalent multifactorial health condition associated with significant morbidity and mortality. Population-based epidemiological studies have revealed an association between systemic autoimmune diseases and deep venous thrombosis (DVT)/VTE. The etiopathogenesis of increased risk of VTE in systemic autoimmune diseases is not entirely clear but multiple contributors have been explored, especially in the context of systemic inflammation and disordered thrombogenesis. Epidemiologic data on increased risk of VTE in patients with primary systemic vasculitides (PSV) have accumulated in recent years and some of these studies suggest the increased risk while patients have active diseases. This could lead us to hypothesize that venous vascular inflammation has a role to play in this phenomenon, but this is unproven. The role of immunosuppressive agents in modulating the risk of VTE in patients with PSV is not yet clear except for Behçet's disease, where most of the studies are retrospective. Sensitizing physicians to this complication has implications for prevention and optimal management of patients with these complex diseases. This review will focus on the epidemiology and available evidence regarding pathogenesis, and will attempt to summarize the best available data regarding evaluation and treatment of these patients.

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Mortality in Behçet's disease

Saadoun

Wechsler

Desseaux

et al. 2010

Arthritis & Rheumatism

268

130

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Objective. To report the long-term mortality in patients with Behçet's disease (BD).Methods. A cohort of 817 patients fulfilling the international criteria for BD from a single center in France were analyzed for causes of death, the standardized mortality ratio (SMR), and the factors associated with mortality.Results. Among the 817 patients with BD, 41 (5%) died after a median followup of 7.7 years, of whom 95.1% were male. The mean ؎ SD age at death was 34.8 ؎ 11.9 years. Main causes of death included major vessel disease (mainly, arterial aneurysm and Budd-Chiari syndrome) (43.9%), cancer and malignant hemopathy (14.6%), central nervous system involvement (12.2%), and sepsis (12.2%). The mortality rate at 1 year and 5 years was 1.2% and 3.3%, respectively. There was an increased mortality among patients ages Conclusion. The overall mortality in our BD cohort was 5% after a median followup of 7.7 years. Male sex, arterial involvement, and the number of flares were associated with mortality in BD.

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Pulmonary artery aneurysms in Behçet syndrome

Cited by 230 publications

References 16 publications

Pulmonary artery aneurysms in Behçet disease

Pulmonary artery aneurysms in Behçet disease

Venous thromboembolism in systemic autoimmune diseases: A narrative review with emphasis on primary systemic vasculitides

Mortality in Behçet's disease

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