Graham Hughes1 and Shirish Sangle2
(1)
The London Lupus Centre, London Bridge Hospital, London, UK
(2)
Louise Coote Lupus Unit, St Thomas’ Hospital, London, UK
Abstract
Hepatic artery thrombosis and liver infarction can occur in APS, as can hepatic venous thrombosis. Budd–Chiari syndrome (thrombosis of the hepatic vein) is a well-known sequel of APS, leading to hepato-splenomegaly (Fig. 16.1). One study has suggested that Hughes Syndrome may be the second commonest cause of Budd–Chiari (second only to lymphoma).
16.1 Liver
Hepatic artery thrombosis and liver infarction can occur in APS, as can hepatic venous thrombosis. Budd–Chiari syndrome (thrombosis of the hepatic vein) is a well-known sequel of APS, leading to hepato-splenomegaly (Fig. 16.1). One study has suggested that Hughes Syndrome may be the second commonest cause of Budd–Chiari (second only to lymphoma).
Abnormal liver function tests are common in APS, and chronic liver ischaemia can lead to liver cirrhosis – a complication of APS whose frequency remains unknown.
16.2 Gut
A number of cases of bowel infarction have been reported, often requiring major surgery. Following the observation that renal artery stenosis is a feature of Hughes Syndrome, Sangle and colleagues in St. Thomas Hospital reported a substantial series of patients with celiac and mesenteric artery stenoses (Fig. 16.2).
Figure 16.1
Hepatic Vein Thrombosis (Budd-Chiari syndrome) and collateral circulation in Hughes syndrome. Picture used courtesy of www.doctorshangout.com
Figure 16.2
Magnetic resonance arteriogram showing superior mesenteric artery stenosis in Hughes syndrome
The clinical features in many cases were those of mesenteric angina – abdominal pain an hour or so after a large meal. (Not all cases were symptomatic, presumably due to the good vascular network of collaterals supplying the gut.)
As with the case of renal artery stenosis, a number of patients with celiac artery stenosis improved with thorough anticoagulation.