BACKGROUND: Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disease associated with arterial and venous thromboembolism and pregnancy complications. There have been several reports of APS with systemic lupus erythematosus (SLE) complicated with aortic dissection. However, none of them has been primary APS, which is APS without SLE.

CASE REPORT: A 42-year-old woman with primary APS and APS nephropathy on warfarin and aspirin therapy presented with coma due to cerebellar hemorrhage. The effect of warfarin was immediately reversed with prothrombin complex concentrate. We performed emergent evacuation of the hematoma, and her level of consciousness improved to normal on postoperative day (POD) 1. She had acute hypertension on arrival, which was resistant to multiple antihypertensives and was stabilized on POD 3. She also had exacerbation of chronic kidney disease after using contrast and prothrombin concentrate complex, and was on temporary renal replacement therapy from POD 3. Aortic dissection was found accidentally on echocardiography on POD 7, and she was subsequently treated medically. She was transferred to the rehabilitation hospital with mild dysarthria and truncal ataxia on POD 59.

CONCLUSIONS: We report the first case in the English literature of primary APS complicated with cerebellar hemorrhage and aortic dissection. Acute hypertension following hemorrhage and exacerbation of APS nephropathy likely triggered the dissection of the aortic wall, the integrity of which might have been compromised by longstanding antiphospholipid antibody and vasa vasorum thrombosis.

Keywords: Antiphospholipid Syndrome, Aortic Diseases, Intracranial Hemorrhages, Aneurysm, Dissecting, Cerebellar Diseases, Coma, Hypertension, Renal Insufficiency, Chronic