Hepatic Artery Stenosis in Antiphospholipid Syndrome

Antiphospholipid syndrome (APS) is characterized by pregnancy morbidity and thrombosis (arterial and venous) in association with antiphospholipid antibodies (aPL). ¹ Recently, a number of publications have demonstrated the presence of arterial stenosis in patients with APS, although the exact etiology remains unclear. ² We report a patient with APS associated with long-standing abdominal symptoms and hepatic artery stenosis.

A 36-year-old female with systemic lupus erythematosus (SLE) and lupus nephritis had been receiving mycophenolate mofetil, low-dose prednisolone, and hydroxychloroquine for the last 2 years. She was a smoker (10 pack years) and had no other conventional cardiovascular risk factors. She had a history of seizures and symptoms suggestive of transient ischemic attacks. The lupus anticoagulant (dilute Russell’s viper venom time [dRVVT]) was positive more than twice 12 weeks apart and magnetic resonance imaging brain scan showed high-density signals suggestive of small vessel ischemia. She was diagnosed with APS and commenced warfarin therapy. She developed abdominal pain over 4 years which was exacerbated after food and was associated with bloating, nausea, urgency to defecate, and weight loss. She did not have vomiting, diarrhea, or rectal bleeding. Her hemoglobin was 10.2 g/dL and hepatic transaminases were within normal limits. Upper gastrointestinal endoscopy, flexible sigmoidoscopy, and colonoscopy were normal and serology was negative for celiac disease antibodies, smooth muscle, and antimitochondrial antibodies. Magnetic resonance imaging angiography of the celiac axis showed a tight stenosis just distal to the origin of the common hepatic artery, with a patent gastroduodenal artery providing a collateral pathway (Figure 1 ).

Figure 1.

Hepatic artery stenosis in a patient with antiphospholipid syndrome.

A number of case reports and series have described arterial stenosis in the renal, ³ celiac, ⁴ and cerebral ⁵ arteries in patients with APS. These stenoses are described as smooth and well delineated, quite different from those seen in patients with atherosclerosis and fibromuscular dysplasia. Some reports have suggested that anticoagulation treatment may improve or prevent further progression of the stenosis. ⁶

The mechanisms underlying the vascular abnormalities in APS are not clear, although autopsy findings in patients with SLE and APS have shown fibroelastic thickening of the intima and thrombosis, ⁷ and a case report of a resected thrombosed superior mesenteric artery showed coexistence of smooth muscle hyperplasia, ⁸ suggesting that intimal and smooth muscle hyperplasia and/or thrombosis individually or together may play a role. Interestingly, the coexistence of APS and arterial aneurysms has also been described, suggesting that this could be an additional vascular presentation of this syndrome. ⁹

Here, we report a patient who fulfilled the classification criteria for APS. ¹⁰ She presented with abdominal symptoms unrelated to her therapy for SLE. Her endoscopies and serology did not explain her gastrointestinal manifestations. Magnetic resonance imaging angiography showed hepatic artery stenosis without any evidence of atherosclerosis (Figure 1) which was assumed to be the probable cause of her symptoms, although the possibility of this being a coincidental finding cannot be excluded.

Hepatic involvement is not uncommon in patients with APS. Previous reports have described hepatic artery aneurysm, ¹¹ hepatic vein thrombosis (Budd–Chiari syndrome), hepatic infarction, and inflammatory and cirrhotic conditions associated with APS/aPL. ¹² To our knowledge, hepatic artery stenosis with abdominal symptoms has not been reported previously.