CT findings of pulmonary artery aneurysms during treatment for Behçet's disease.

Tunacı, Mehtap; Özkorkmaz, Berrin; Tunacı, Atadan; Gül, Ahmet; Engin, Gülgün; Acunaş, Bülent

doi:10.2214/ajr.172.3.10063870

Cited by 127 publications

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References 11 publications

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“…Aneurysms were most frequently located in the lobar artery in the right lower lobe (35%), followed by the lobar artery in the left lower lobe (19%) and right main pulmonary artery (17%), and 33% of aneurysms had partial or total thrombus within the aneurysm itself. 109 The present study showed similar results to those published by Tunaci et al 109 Another important finding of Tunaci et al 109 was complete or partial PAA resolution in response to immunosuppressive treatments: 76% of PAAs completely disappeared in 3-42 (mean: 21) months and 24% of PAAs regressed in 4-28 (mean: 17) months after treatment. 109 It was reported that the largest aneurysm was 7 cm, 109 and the mean diameter of aneurysms was 2.3±1.1 cm.…”

Section: Discussionsupporting

confidence: 91%

“…109 The present study showed similar results to those published by Tunaci et al 109 Another important finding of Tunaci et al 109 was complete or partial PAA resolution in response to immunosuppressive treatments: 76% of PAAs completely disappeared in 3-42 (mean: 21) months and 24% of PAAs regressed in 4-28 (mean: 17) months after treatment. 109 It was reported that the largest aneurysm was 7 cm, 109 and the mean diameter of aneurysms was 2.3±1.1 cm. 122 The present study, however, revealed much larger dimensions of PAAs with a mean diameter of 4.0 cm.…”

Section: Discussionsupporting

confidence: 91%

“…2 Erkan et al 2 reported that PAAs are most often located in the right lower lobar arteries, followed by bilateral main pulmonary branches. Tunaci et al 109 proposed that the pulmonary arteries of the lower lobe were the most common site of involvement, and a mural thrombus was observed in 85% of PAAs. Aneurysms were most frequently located in the lobar artery in the right lower lobe (35%), followed by the lobar artery in the left lower lobe (19%) and right main pulmonary artery (17%), and 33% of aneurysms had partial or total thrombus within the aneurysm itself.…”

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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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

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Pulmonary artery aneurysms in Behçet disease

Yuan

2014

J. vasc. bras.

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“…As with pulmonary artery aneurysm (PAA), a combination of cyclophosphamide and methylprednisolone is most frequently prescribed for patients with severe vasculitis, but in patients with BD, the PAA may become smaller or disappear with medical treatment. [12] Occlusion of the hepatic portion of the IVC, major hepatic vein, or both can lead to BCS, and the prevalence of BCS in BD is 3.2%. Behçet's disease is an uncommon cause of BCS, and only 56 cases of BD complicated by BCS have been published.…”

Section: Discussionmentioning

confidence: 99%

Intracardiac Thrombosis and Coronary-to-Pulmonary Artery Fistula with Pulmonary Embolism and Budd-Chiari Syndrome in Behçet's Disease: A Case Report

Aksu

Öz

2013

Turk J Rheumatol

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Behçet Hastalığı (BH) multisistemik, kronik ve inflamatuvar vaskülit tablosudur. Behçet hastalığı olan 29 yaşında erkek hasta dispne ve göğüs ağrısı ile kliniğimize başvurdu. Hastanın fizik muayenesinde sol parasternal -makine sesine benzer-bir üfürüm ve abdominal asit saptandı. Hastanın elektrokardiyografi ve göğüs röntgen sonuçları normaldi. Toraks bilgisayarlı tomografide (BT) kronik pulmoner emboli izlendi. Abdominal BT'de BuddChiari sendromu (BCS) ile uyumlu olarak hepatik ven ve inferior vena kavada trombüs izlendi. İki boyutlu transtorasik ekokardiyografide sağ atriyumdan sağ ventriküle uzanım gösteren kitle izlendi. Suprasternal pencerede arkus aortu ve pulmoner arteri çevreleyen anormal akım tespit edildi. Koroner anjiyografide sol ana koroner arter ile pulmoner arter arasında fistül oluşumu gözlendi. Antiinflamatuvar ilaçlar ve heparin tedavisi başlandı. Altıncı ayda pulmoner emboli ve kalp içi trombüste tam düzelme izlendi. Bu yazıda BH olan bir hastada kalp içi trombüs, Budd-Chiari sendromu ve pulmoner emboli ile ilişkili koroner-pulmoner arter fistülü birlikteliği sunuldu.Anahtar sözcükler: Behçet hastalığı; fistül; pulmoner emboli; trombüs.Behçet's disease (BD) is a multisystemic, chronic and inflammatory vasculitis presentation. A 29-year-old male patient with BD was admitted to our clinic with dyspnea and chest pain. On his physical examination, a left parasternal murmur resembling the sound of a machine was detected along with abdominal ascites. The patient's electrocardiography and chest X-ray were normal. Thoracic computed tomography (CT) revealed a chronic pulmonary embolism. On abdominal CT, a thrombus was detected in the hepatic vein and inferior vena cava, which was consistent with Budd-Chiari syndrome (BCS). Twodimensional transthoracic echocardiography demonstrated a mass on the right atrium protruding into the right ventricle. On the suprasternal view, abnormal flow surrounding the arcus aorta and pulmonary artery was detected. Coronary angiography showed a fistula formation between the left main branch and pulmonary artery. A treatment with anti-inflammatory drugs and heparin was initiated. Complete recovery of the pulmonary embolism and intracardiac thrombosis were observed at six months. In this article, we present a BD patient with intracardiac thrombosis and coronary-to-pulmonary fistula associated with a pulmonary embolism and BCS.

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“…Although not clearly established as yet, there is conflicting data concerning the preventive potential immunosuppressive therapy on vascular diseases and complications [39]. Immunosuppressive drug therapy alone or in combination with steroids is advised during early stages of BD before the development of irreversible damage to the arterial wall [39].…”

Section: Treatment In Venous Involvementmentioning

confidence: 99%

Vascular Manifestations of Behçet’s Disease

Demïrtürk¹,

Tünel²,

Alemdaroğlu³

2017

Behcet's Disease

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Behçet's disease (BD), a very morphologically diverse systemic disease, may involve the vascular system. The venous system is the most frequently attacked vessel system.The arterial system, when involved, increases the severity and morbidity of Behçet's disease. Cardiac involvement, although rare, can be very subtle and in itself increases the mortality. Vasculitis is the hallmark pathology resulting in occlusion, aneurysms, or both.Vascular involvement may be very challenging in all phases of treatment beginning from diagnosis till recovery and remission.

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CT findings of pulmonary artery aneurysms during treatment for Behçet's disease.

Cited by 127 publications

References 11 publications

Pulmonary artery aneurysms in Behçet disease

Pulmonary artery aneurysms in Behçet disease

Intracardiac Thrombosis and Coronary-to-Pulmonary Artery Fistula with Pulmonary Embolism and Budd-Chiari Syndrome in Behçet's Disease: A Case Report

Vascular Manifestations of Behçet’s Disease

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