Antiphospholipid syndrome presenting as episodic limb ischaemia.

Abstract

A child presented with ischaemic episodes of his left leg from the age of 2 months. He was found to have raised anticardiolipin antibodies so was started on low dose aspirin. At a three year follow up he was asymptomatic with a normal anticardiolipin antibody level while taking aspirin daily, probably for life. (Arch Dis Child 1996;75:342-343) Keywords: antiphospholipid syndrome, acute limb ischaemia.The antiphospholipid syndrome was first described in 1983 by Hughes et al in patients with systemic lupus erythematosus (SLE) Examination revealed a well looking baby boy with weight and head circumference on the 50th centile and length on the 97th centile. His limbs were not mottled or cyanosed. His femoral pulses and four-limb blood pressure measurements were normal. The rest of the examination was entirely normal.Since these episodes sounded ischaemic rather than being associated with temperature or autonomic immaturity as commonly seen in babies, we undertook further studies. Ultrasonography excluded a pelvic mass, and neither immersion in cold water nor compressing his femoral vessels precipitated similar episodes. Doppler studies of the femoral arteries and veins, echocardiography, and electrocardiography were all normal. His blood count was normal except for a platelet count of 600 x 1 09/l. The erythrocyte sedimentation rate (ESR) was 3 mm/hour, there was a normal clotting screen, a negative autoimmune profile, and no anti-DNA antibodies were detected. IgG anticardiolipin antibody was raised at 14.9 AEU (the upper range of normal being 7), with normal levels of IgM anticardiolipin antibody. However, by this time the episodes had stopped so the significance of the raised IgG anticardiolipin was questioned. The test was repeated at 9 months of age when the IgG anticardiolipin had risen to 26.5 AEU. We therefore diagnosed antiphospholipid syndrome without an obvious precipitating cause.

Familial antiphospholipid syndrome presenting as bivessel arterial occlusion in a 17-year-old girl

“…Paediatric APS extremely rarely presents with acute limb ischaemia. Our literature review came up with only one report of acute distal ischaemia as a presentation of antiphospholipid syndrome in a child [5], [6].…”