Zakaria, N.; Wendon, J.; Devlin, J.
April 2003
Gut;Apr2003 Supplement 1, Vol. 52, pA35
Academic Journal
Background and Aims: Antiphospholipid syndrome (APS) is increasingly recognised to have a wide range of clinical manifestations, although involvement from this disorder within the liver or its vasculature is poorly identified. Patients and Methods: 49 consecutive patients with a liver disorder investigated between 1994 and 2002 who fulfilled the diagnostic criteria for APS were studied. Patients were followed up for a median of 10 months (range 6-94 months). Results: Of the 49 cases, 21 were male, 28 female. Median age at presentation was 42 (range 17-67). 29 patients were north European (60%), 5 (10%) Mediterranean, 8 (16%) Afro-Caribbean, and 7 (14%) Asian. 36 patients had primary APS (80%), 7 had APS associated with SLE (14%) and 3 associated with other autoimmune diseases (6%). 3 patients had "equivocal" APS. A diagnosis of APS had been made in only 2 of 49 (4%) symptomatic cases prior to referral. A wide range of clinical and pathological findings involving the liver parenchyma and its vascular compartments were identified. 6 (12%) had either thrombosis or arteritis of the hepatic arterial tree. Porto-mesenteric thrombosis was present in 13 cases (27%) and nodular regenerative hyperplasia in 7 (14%). In 9 patients, Budd Chiari syndrome (6), veno-occlusive disease (2) or congestion secondary to right ventricular failure (1) was diagnosed. Autoimmune hepatitis was diagnosed in 9 patients. Associated non-hepatic clinical manifestations included vasculitis (10 (20%)), thromboses (8 (16%)) and unexplained myocardial dysfunction (5 (10%)). Anticardiolipin antibodies were detected in 48 of 49 (98%) and Lupus anticoagulant in 14 of 36 (39%) of the patients tested. Complement C4 levels were Iow in 22 of 33 (67%) patients tested. 7 of 49 (14%) had SLE. 11 underwent transplantation for APS related liver disease (Budd-Chiari syndrome 3, hepatic artery thrombosis 3, NRH 3, autoimmune hepatitis 1, veno-occlusive disease 1). 4 of the 49 patients died. Conclusion: The...


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