Pulmonary Arterial Aneurysms in Behçet's Syndrome: A Report of 24 Cases

Hamuryudan, Vedat; Yurdakul, S; Moral, F. de la Calle del; Numan, Fürüzan; Tüzün, Hasan; Tuzuner, Nukhet; Mat, Cem; Tüzün, Yalçın; Özyazgan, Yılmaz; Yazıcı, Hasan

doi:10.1093/rheumatology/33.1.48

Cited by 278 publications

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“…Generally speaking, large-vessel disease and neurologic involvement are the main causes of mortality in BD (40,41). In our population, no patients had large-vessel disease.…”

Section: Seventeen-year Study Of Behçet's Disease In Italymentioning

confidence: 60%

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Salvarani¹,

Pipitone²,

Catanoso³

et al. 2007

Arthritis & Rheumatism

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Objective. To investigate the epidemiology and clinical course of Behçet's disease (BD) over a 17-year period in a defined area of northern Italy. Methods. All patients with incident BD diagnosed over a 17-year period (from January 1, 1988 to December 31, 2004) living in the Reggio Emilia area were identified through the following sources: physicians at Reggio Emilia Hospital, medical practitioners, and community-based specialists. We identified all patients registered in a centralized index and in the Reggio Emilia district database for rare diseases. Patients were followed up from the time of diagnosis until either their death or April 1, 2005. Results. Eighteen patients (9 men and 9 women) had complete BD. Mean ؎ SD age at diagnosis was 33 ؎ 7 years. The incidence rate of BD was 0.24 per 100,000. The prevalence of BD on January 1, 2005 was 3.8 per 100,000. No patients died during the followup period. Although all patients developed oral ulceration during the disease course, 22.2% had no oral lesions at disease onset. Eye disease occurred in 55.6%. Ocular disease was more common in men and appeared at disease onset or within the first few years of disease onset (median 3 years). Only 1 patient had loss of useful vision in at least 1 eye at the end of followup. In all affected patients, visual acuity improved once treatment was started. Conclusion. This population-based study is the first to report the prevalence and incidence of BD in Italy. In Italian patients, BD is nonfatal and the prognosis of eye disease is good.

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“…Generally speaking, large-vessel disease and neurologic involvement are the main causes of mortality in BD (40,41). In our population, no patients had large-vessel disease.…”

Section: Seventeen-year Study Of Behçet's Disease In Italymentioning

confidence: 60%

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Salvarani¹,

Pipitone²,

Catanoso³

et al. 2007

Arthritis & Rheumatism

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“…Neurologic involvement (2) group 2 placebo patients compared with 1 of 12 group 2 AZA patients who had eye disease of >2 years duration at the time of entry into the AZA trial (log rank 2 = 1.5, P = 0.21) ( Figure 7). Extraocular involvement.…”

Section: Resultsmentioning

confidence: 99%

Azathioprine in Behçet's syndrome. Effects on long‐term prognosis

Hamuryudan¹,

Özyazgan²,

Hizli³

et al. 1997

Arthritis & Rheumatism

249

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Objective. To assess the effect of azathioprine (MA) treatment on long-term prognosis in Behget's syndrome.Methods. Patients (all male) who took part in a double-blind, placebo-controlled trial of AZA a mean f SD of 94 f 10 months previously were reevaluated.Results. The emergence of blindness (log rank 2 = 5.6, P = 0.02) and a 2-line drop in the visual acuity of the right eye (log rank 2 = 5.9, P = 0.015) occurred significantly more frequently among the patients originally allocated to the placebo group compared with patients who originally received M A , despite posttrial treatment for patients in both groups when needed. There was also a trend toward more frequent occurrence of extraocular complications in the placebo group. The beneficial effect of AZA was especially pronounced among patients who had eye involvement of short duration prior to their entry into the trial.Conclusion. Early treatment with M A tends to favorably affect the long-term prognosis of Behget's syndrome.

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“…Histopathological observations revealed pulmonary vasculitis involving all layers of pulmonary arteries and veins, 3 resulting in thrombosis, stenosis, aneurysm formation and rupture. Mononuclear inflammatory cells, predominantly lymphocytes, are responsible for inflammatory infiltration in and around the vessel wall.…”

Section: Discussionmentioning

confidence: 99%

“…121 In the early years, short-term survival of patients with PAAs associated with Behçet's disease was only 50%. 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: Discussionmentioning

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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Pulmonary Arterial Aneurysms in Behçet's Syndrome: A Report of 24 Cases

Cited by 278 publications

References 0 publications

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Azathioprine in Behçet's syndrome. Effects on long‐term prognosis

Pulmonary artery aneurysms in Behçet disease

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