Vasculo-Behçet’s disease with non-traumatic subcapsular hematoma of the kidney and aneurysmal dilatations of the celiac and superior mesenteric arteries

Yokota, Kumiko; Akiyama, Yuji; Sato, Kojiro; Shindo, Yasufumi; Yoshida, Yasuhiko; Miyoshi, Fumito; Akiba, Haruhiko; Nakajima, Kiyokazu; Asanuma, Yu; Mimura, Toshihide

doi:10.1007/s10165-008-0091-7

Cited by 7 publications

(13 citation statements)

References 12 publications

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“…In 25 cases, the multisystem disorder involved oral aphthosis, 5 – 22 genital aphthosis, 5 , 6 , 9 , 10 , 12 – 17 , 19 , 21 , 22 skin lesions, 7 , 10 , 12 , 14 , 16 , 19 ocular lesions, 6 – 9 , 14 , 15 , 17 , 19 – 21 , 23 vascular involvement, 5 , 9 , 11 , 15 , 17 , 24 and positive pathergy test. 6 , 15 , 16 , 23 The BD symptoms do not necessarily involve or manifest simultaneously. Patient characteristics are summarized in Table 2 .…”

Section: Resultsmentioning

confidence: 99%

Spontaneous Intra-Abdominal Hemorrhage Due to Rupture of Jejunal Artery Aneurysm in Behcet Disease

Wei

Zhao

et al. 2015

Medicine

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Rupture of jejunal artery aneurysm is a very rare event resulting in life-threatening hemorrhage in Behcet disease (BD). We report a case of ruptured jejunal artery aneurysm in a 35-year-old patient with BD. The patient had a 1-year history of intermittent abdominal pain caused by superior mesenteric artery aneurysm with thrombosis. Anticoagulation treatment showed a good response. Past surgical history included stenting for aortic pseudoaneurysm. On admission, the patient underwent an urgent operation due to sudden hemorrhagic shock. Resection was performed for jejunal artery aneurysm and partial ischemia of intestine. The patient was diagnosed with BD, based on a history of recurrent oral and skin lesions over the past 6 years. Treatment with anti-inflammatory medications showed a good response during the 8-month follow-up.An increased awareness of BD and its vascular complications is essential. Aneurysms in BD involving jejunal artery are rare, neglected and require proper management to prevent rupture and death. To our knowledge, this is the first reported case of jejunal artery aneurysm caused by BD.

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Section: Resultsmentioning

confidence: 99%

Spontaneous Intra-Abdominal Hemorrhage Due to Rupture of Jejunal Artery Aneurysm in Behcet Disease

Wei

Zhao

et al. 2015

Medicine

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“…Moreover, nephrotic syndrome might be present in over 83% of patients with amyloidosis, and renal failure is common at the time of diagnosis [7,8,10]. Other case reports also describe some rare forms of renal involvement in Behçet's disease, such as perirenal haematoma [12], renal microinfarction [13] and nontraumatic subcapsular kidney haematoma [14].…”

Section: Discussionmentioning

confidence: 97%

A severe renal bleeding as a complication of coumarin therapy in Behçet's disease

Remková

Milatová²,

Fuchsbergerová³

2010

Blood Coagulation &Amp; Fibrinolysis

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Behçet's disease is a systemic inflammatory disease, which predisposes patients to venous or arterial thrombosis. We report a case of 41-year-old patient who presented for several years with recurrent fevers, arthralgias, episodes of skin eruptions and recurrent bilateral deep venous ileofemoral thromboses, extending into the inferior vena cava, despite the oral anticoagulant therapy. Additionally, he also reported recurrent aphthous oral and genital lesions, and eye problems. A laboratory picture of chronic inflammation, normocytic anaemia and a finding of lupus anticoagulant/antiphospholipid antibodies were observed. PET raised a high suspicion of vasculitic PTT-lupus anticoagulant process, involving the large vessels. During an anticoagulant therapy by warfarin (dose in upper limit of therapeutic range), the left kidney had to be removed because of acute retroperitoneal haemorrhage. On histologic examination, the picture of nephritis was described. A detailed retrospective reevaluation of complex history as well as other clinical findings strongly raised the suspicion of a systemic vasculitic syndrome, such as Behçet's disease, accompanied by antiphospholipid syndrome. The patient responded well to the combination of colchicine and anticoagulant therapy by low-molecular-weight heparin. The episodes of fever, skin eruptions and other symptoms disappeared. We assume that this is a case of an unusual course of Behçet's disease presenting with a rare kidney involvement. Kidney disorder complicated the oral anticoagulant therapy for recurrent venous thromboses and led to the excessive renal bleeding, requiring nephrectomy. Recognition that nephritis is associated with Behçet's disease may be useful to prevent severe renal bleeding in relation to anticoagulant therapy.

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“…Patients with vasculo-Behcet are at risk of developing recurrent vascular lesions and are prone for progressive multifocal vessel related complications [ 2 ]. Aorta is the most susceptible site to aneurysm, followed by the pulmonary artery, femoral artery, subclavian artery, cervical artery and popliteal artery [ 6 ]. Involvement of the coronary and renal arteries is very rare [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…There was also actively bleeding ruptured aneurysm on CT images. Zhang et al reported that there was no BD in 165 cases as a cause of non-traumatic renal bleeding [ 6 ]. To our knowledge, there have been no reports of BD both with Budd-Chiari syndrome and multiple microaneurysms in the kidney presented with renal hematoma, and superselective catheterization and embolization of actively bleeding aneurysm, with the same patient.…”

Section: Discussionmentioning

confidence: 99%

Budd-chiari syndrome and renal arterial neurysms due to behcet disease: a rare association

et al. 2015

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Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm.

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Vasculo-Behçet’s disease with non-traumatic subcapsular hematoma of the kidney and aneurysmal dilatations of the celiac and superior mesenteric arteries

Cited by 7 publications

References 12 publications

Spontaneous Intra-Abdominal Hemorrhage Due to Rupture of Jejunal Artery Aneurysm in Behcet Disease

Spontaneous Intra-Abdominal Hemorrhage Due to Rupture of Jejunal Artery Aneurysm in Behcet Disease

A severe renal bleeding as a complication of coumarin therapy in Behçet's disease

Budd-chiari syndrome and renal arterial neurysms due to behcet disease: a rare association

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