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

Batur A, Dorum M, Yüksekkaya HA, Koc O - Pan Afr Med J (2015)

Bottom Line: Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Yuzuncuyil University Dursun Odabas Medical Center, Van, Turkey.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

VRT images of CT angiography demonstrates vena cava inferior thrombosis (long arrows) with venous collaterals (short arrows)
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Figure 0001: VRT images of CT angiography demonstrates vena cava inferior thrombosis (long arrows) with venous collaterals (short arrows)

Mentions: Sixteen year-old male presenting with severe abdominal pain and swelling on the left quadrant of the abdomen. In the history, when he was 6 years old investigated with abdominal pain, ascites and fever. Doppler ultrasonography and BT angiography demonstrated heterogenity in liver parenchyma, hepatic vein and vena cava inferior thrombosis with venous collaterals (Figure 1). Acute phase markers were elevated. Screening revealed that the first allele of MEFV gene V726 A mutation was positive. His Factor 5 Leiden G1691A mutation, MTHFR mutation, prothrombin 20120A, lupus anticoagulan screens were negative. Protein C-S and homocysteine levels were in normal range. He was diagnosed Budd-Chiari Syndrome with Familial Mediterranian Fever. Colchicine treatment had been started. At the age of 8 years he presented with iliopsoas muscle hematom. Skin rash appeared during the hospital stay. Skin biopsy was consistent with leukocytoclastic vasculitis. Pathergy test was 6/5 positive. Eye examination was normal. They thought the diagnosis of Behcet´s disease, and continued to colchisine therapy. On his family history there was no kindship between the parents. His aunt was died from chronic renal failure (its etiology was not explained). In the physical examination; the height was 170cm (%25-50), weight was 55kg (%10-25), overall condition was poor, hypotensive, tachypneic, tachycardic. There were hepatosplenomegaly and swelling on the left quadrant of the abdomen. The other system examinations were normal.


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

Batur A, Dorum M, Yüksekkaya HA, Koc O - Pan Afr Med J (2015)

VRT images of CT angiography demonstrates vena cava inferior thrombosis (long arrows) with venous collaterals (short arrows)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4594976&req=5

Figure 0001: VRT images of CT angiography demonstrates vena cava inferior thrombosis (long arrows) with venous collaterals (short arrows)
Mentions: Sixteen year-old male presenting with severe abdominal pain and swelling on the left quadrant of the abdomen. In the history, when he was 6 years old investigated with abdominal pain, ascites and fever. Doppler ultrasonography and BT angiography demonstrated heterogenity in liver parenchyma, hepatic vein and vena cava inferior thrombosis with venous collaterals (Figure 1). Acute phase markers were elevated. Screening revealed that the first allele of MEFV gene V726 A mutation was positive. His Factor 5 Leiden G1691A mutation, MTHFR mutation, prothrombin 20120A, lupus anticoagulan screens were negative. Protein C-S and homocysteine levels were in normal range. He was diagnosed Budd-Chiari Syndrome with Familial Mediterranian Fever. Colchicine treatment had been started. At the age of 8 years he presented with iliopsoas muscle hematom. Skin rash appeared during the hospital stay. Skin biopsy was consistent with leukocytoclastic vasculitis. Pathergy test was 6/5 positive. Eye examination was normal. They thought the diagnosis of Behcet´s disease, and continued to colchisine therapy. On his family history there was no kindship between the parents. His aunt was died from chronic renal failure (its etiology was not explained). In the physical examination; the height was 170cm (%25-50), weight was 55kg (%10-25), overall condition was poor, hypotensive, tachypneic, tachycardic. There were hepatosplenomegaly and swelling on the left quadrant of the abdomen. The other system examinations were normal.

Bottom Line: Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Yuzuncuyil University Dursun Odabas Medical Center, Van, Turkey.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus