2002, Number 3
<< Back Next >>
Rev Med Hosp Gen Mex 2002; 65 (3)
Budd-Chiari syndrome secondary to primary antiphospholipid antibodies syndrome: Report of a case and review of the literature
Lastra CG, Soriano RJ, Carrera GE, Guevara LB, Durán PMA
Language: Spanish
References: 12
Page: 164-167
PDF size: 126.58 Kb.
ABSTRACT
A 20 year old woman presented with symmetrical lower limb edema and abdominal enlargement, the physical exam revealed jaundice and ascites. Laboratory results included positive VDRL test and circulating antiphospholipid antibodies (aPL). The patient developed progressive respiratory failure and died. The autopsy revealed inferior vena cava and hepatic vein thrombosis, a mural thrombi in the right atrium, left pulmonary artery thrombi and an old pulmary infarction in the same side. Budd-Chiari syndrome secondary to primary antiphospholipid syndrome was established, since no other autoimmune or connective tissue disease was found. The hepatic complications of the antiphospholipid syndrome includes Budd-Chiari syndrome, veno-oclussive disease, nodular regenerative hyperplasia, transient elevation of liver enzymes and infarction. The pathogenesis of the thrombosis is not well understood. Some authors thought that antiphospholipid syndrome is the second cause of non-tumorous Budd-Chiari syndrome.
REFERENCES
Okuda K, Kage M, Shrestha SM. Proposal of a new nomenclature for Budd-Chiari syndrome: Hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion. Hepatology 1998; 28: 1191-1198.
Wyatt HA, Mowat AP, Layton M. Paroxismal nocturnal haemoglobinuria and Budd-Chiari syndrome. Arch Dis Child 1995; 72: 241-242.
Walsh MM, Hytiroglou P, Thung SN. Epithelioid Hemangioendothelioma of the liver mimicking Budd-Chiari syndrome. Arch Pathol Lab Med 1998; 122: 846-848.
Tilanus HW. Budd-Chiari syndrome. Br J Surg 1995; 82: 1023-1030.
Alarcón-Segovia D, Sánchez-Guerrero J. Primary antiphospholipid syndrome. J Rheumatol 1989; 16: 482-488.
Asherson RA, Khamashta MA, Ordi-Ros J et al. The “primary” antiphospholipid syndrome: Major clinical and serological features. Medicine 1989; 68: 366-374.
Asherson RA, Khamashta MA. The hepatic complications of the antiphospholipid antibodies. Clin Exp Rheumatol 1991; 9: 341-344.
Dudley DJ, Mitchell MD, Branch DW. Pathophysiology of antiphospholipid antibodies: Absence of prostaglandin-mediated effects on cultured endothelium. Am J Obstet Gynecol 1990; 162: 953-9.
Pelkonen P, Simell O, Rasi V et al. Venous thrombosis associated with lupus anticoagulant and anticardiolipin antibodies. Acta Paedit Scand 1988; 77: 767-772.
Ludwig J, McGill DB. Classification of hepatic venous outflow obstruction: Ambiguous terminology of the Budd-Chiari syndrome. Mayo Clin Proc 1990; 65: 51-55.
Pelletier S, Landi B, Piette JC et al. Antiphospholipid syndrome as the second cause of non-tumorous Budd-Chiari syndrome. J Hepatology 1994; 21: 76-80.
Saca LF, Szer IL, Henar E et al. Budd-Chiari syndrome associated with antiphospholipid antibodies in a child: Report of a case and review of the literature. J Rheumatol 1994; 21: 545-548.