Fatal haemoptysis due to pulmonary artery aneurysm in Behçet's disease

Gebitekin, Cengiz; Yilmaz, Mehmet; Şenkaya, İşık; Saba, Davit; Sagdic, Kadir; Özer, Gözde Kirli

doi:10.1016/s1078-5884(97)80027-5

Cited by 12 publications

(10 citation statements)

References 14 publications

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“…One of the most common forms of great artery involvement in BD is pulmonary artery aneurysm. There are several reports of pulmonary artery aneurysm and massive hemoptysis in BD including our case 14‐16 …”

Section: Discussionmentioning

confidence: 68%

“…There are several reports of pulmonary artery aneurysm and massive hemoptysis in BD including our case. [14][15][16] Great vessel involvement in BD most frequently affects the venous system, usually as venous thrombosis in superior vena cava, inferior vena cava, or hepatic veins, thus patients may present with symptoms and signs of superior vena cava (SVC) syndrome, 17 or Budd-chiari syndrome. 18,19 Every part of the aorta and its branches may become aneurysmal including carotid arteries, 20 abdominal aorta, 21 and more frequently femoral arteries, iliac arteries, subclavian, popliteal, or rarely brachial arteries.…”

Section: Discussionmentioning

confidence: 99%

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Cardiac and Great Vessel Involvement in “Behcet's Disease”

Marzban

Mandegar

Karimi

et al. 2008

Journal of Cardiac Surgery

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Behcet's disease is a multisystem disorder and classified as "vasculitic syndrome with a wide variety of clinical manifestations." Cardiac involvement is very rare but can occur with different presentations including: pericarditis, cardiomyopathy, endocarditis, endomyocardial fibrosis, intracavitary thrombosis, and coronary artery disease. Great vessel involvement is more common. Recurrent Phlebitis, commonly involving large vessels (superior vena cava, inferior vena cava, hepatic veins) and cerebral veins are the sole presentation in this regard. Arterial involvement is expressed by aneurysm or pseudoaneurysmal formation. Due to the wide variety of cardiovascular manifestations and the resulting high mortality, cardiac surgeons should be familiar with this disease. In this paper we review the articles and introduce our four cases presenting with aneurysm of ascending aorta with free aortic insufficiency, aneurysm of descending aorta, pulmonary artery aneurysm, and pseudoaneurysm of aortic arch. doi: 10.1111/j. 1540-8191.2008.00607.x (J Card Surg 200823:765-768) Behcet's disease (BD) was first discovered in 1937 by Hulusi Behcet. It is an autoimmune disease and is classified as a vasculitic syndrome. This disease is a multisystem disorder with a wide variety of clinical manifestations including skin, eye, musculoskeletal, neurologic, and cardiovascular presentations. 1,2 The spectrum of cardiac diseases may include pericarditis, coronary artery stenosis or aneurysm, myocarditis, cardiomyopathy, congestive heart failure, valvular pathology, endocarditis or endocavitary thrombosis, aneurysm of aorta and its branches, pulmonary artery aneurysm, or venous thrombosis. Due to its wide variety of pathologies, disease presentation may vary in each case. CASE REPORT Case 1A 24-year-old man, a known case of BD, under followup by the rheumatology clinic in our center, developed exertional chest pain a few weeks prior to presentation to our ward. His workup, including chest xray (CXR), computed tomography (CT) scan, transthoracic echocardiography, and aortography, showed cardiomegaly, severe aortic insufficiency, and a huge aneurysm of ascending aorta as is shown in (Fig. 1). aortic valve no. 23. The patient had a smooth postoperative course and an uneventful recovery, but 20 days postoperative the patient was operated on once again Address for correspondence: Mehrab Marzban, M.D., Tehran Heart Center, Tehran, Iran. Fax: +98-21-88029256; e-mail: mehrabmarzban2007@yahoo.com due to massive pericardial effusion resulting from subxiphoid drainage. Now, two years postoperative, the patient is on maintenance prednisolone therapy. Case 2A 50-year-old woman, a known case of BD with cutaneous and ocular manifestation on prednisolone and colchicine, presented to our center with a history of upper abdominal pain, mild dysphagia, and weight loss for a few weeks. Her workup, including CXR, echocardiography, CT scan, and aortogram, showed a saccular aneurysm of descending aorta, just above the diaphragm (Fig. 2). The patient...

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Section: Discussionmentioning

confidence: 68%

Section: Discussionmentioning

confidence: 99%

Cardiac and Great Vessel Involvement in “Behcet's Disease”

Marzban

Mandegar

Karimi

et al. 2008

Journal of Cardiac Surgery

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“…The indications for conservative treatment in severe patients were extensive PAA locations, 55,97 failed interventional embolization 95 and patient reluctance to undergo interventional embolization or surgical operations. 29,68,114 Hematopoietic stem cell transplantation 86 and tumor necrosis factor-α plus infliximab 25 were indicated for patients refractory to conventional immunosuppressive therapy.…”

Section: Resultsmentioning

confidence: 99%

“…72 Patients with rapidly expanding pulmonary artery (pseudo)aneurysms 24,26,107 or with recurrent PAA following successful percutaneous embolization 24 usually warranted surgical operations; while patients with uncontrolled massive hemoptysis with impending PAA rupture often required an emergency operation. 55,59,106,110 A total of 9 patients received intervention/surgery for the treatment of PAA on an urgent basis including 1 interventional embolization 41 and 8 open thoracic operations. 24,35,55,85,110,115,118 In all 9 of these patients an urgent procedure was indicated for massive hemoptysis, and 4 (44.4%) patients of whom suffered from a PAA rupture.…”

Section: Resultsmentioning

confidence: 99%

“…55,59,106,110 A total of 9 patients received intervention/surgery for the treatment of PAA on an urgent basis including 1 interventional embolization 41 and 8 open thoracic operations. 24,35,55,85,110,115,118 In all 9 of these patients an urgent procedure was indicated for massive hemoptysis, and 4 (44.4%) patients of whom suffered from a PAA rupture. 55,110,115,118 Patients were followed-up for 22.5±33.5 (range: Outcomes were reported for 175 patients and comparison of outcomes across different treatments revealed that conservatively treated patients were prone to suffer from PAA progression, that interventional embolization was associated with higher risks of recurrence and reintervention for PAAs, and that surgically treated patients had the highest overall and early mortality rates.…”

Section: Resultsmentioning

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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Transcatheter embolotherapy of pulmonary artery aneurysms as emergency treatment of hemoptysis in Behcet patients: experience of a referral center and a review of the literature

et al. 2018

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Hemoptysis is a life-threatening complication of Behcet's disease that is likely related to pulmonary artery aneurysm (PAA). Vascular interventional radiology may offer effective emergency therapeutic option, but has not been thoroughly investigated in this setting. A case series of a French referral center for hemoptysis combined with a literature review of case reports was conducted. Between 1995 and 2016, 12 patients were referred to our center for hemoptysis revealing or complicating the course of Behcet's disease. Pulmonary artery aneurysm (PAA) was the mechanism of hemoptysis in ten patients, nine of whom were treated by a transcatheter embolotherapy. Combining an additional 8 case reports from the literature, 17 patients treated by transcatheter embolotherapy for PAA were analyzed. The duration of the course of Behcet's disease was 22 months [IQR 3-45] at the time of PAA diagnosis. Transcatheter embolotherapy of PAA was successful for immediately controlling hemoptysis in all patients, without major complication except for one. Hemoptysis recurred in seven patients (41%) within 5 months [IQR 1-12]. The use of coils for transcatheter embolotherapy was associated with hemoptysis recurrence. A bronchosystemic hypervascularization related to the previously occluded PAA was the main mechanism of bleeding recurrence, leading to bronchosystemic artery embolization in four patients and surgery in two patients. Behcet's disease-related hemoptysis is mainly due to PAA. Transcatheter embolotherapy should be considered as the first-line emergency treatment for PAA-related hemoptysis, in association with the immunosuppressive regimen. Hemoptysis may recur in half of the cases, involving preferentially a bronchosystemic arterial mechanism.

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Fatal haemoptysis due to pulmonary artery aneurysm in Behçet's disease

Cited by 12 publications

References 14 publications

Cardiac and Great Vessel Involvement in “Behcet's Disease”

Cardiac and Great Vessel Involvement in “Behcet's Disease”

Pulmonary artery aneurysms in Behçet disease

Transcatheter embolotherapy of pulmonary artery aneurysms as emergency treatment of hemoptysis in Behcet patients: experience of a referral center and a review of the literature

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