Vascular Injury by Arterial Hypertension in Anti-Phospholipid Antibody Syndrome

Abstract

Antiphospholipid Syndrome (APS) is defined as the presence of recurrent arterial or venous thrombosis and/or pregnancy morbidity and persistently elevated levels of antiphospholipid antibodies (aPLs) [1]. APS is classified as being primary if autoimmunity is negative, and secondary when it occurs in the course of an autoimmune disease, most often systemic lupus erythematosus [2]. In the original description of the syndrome made by Hughes, arterial hypertension was a common feature that was often associated with livedo reticularis [3]. Thrombosis in vessels of all sizes of the renal vasculature may be responsible for a wide range of renal diseases including systemic hypertension, renal artery stenosis, renal infarction, APS nephropathy, thrombotic microangiopathy and renal vein thrombosis [4]. The prevalence of hypertension (which ranges from mild to severe) in APS is significantly higher in the presence of renal involvement (57.1% versus 22.6% in Sinico's cohort [5]), and the kidney may be considered both the ‘culprit’ and the ‘victim’ of hypertension in APS, as occurs in other forms of hypertension. We present a case of primary APS with multiple organ involvement, kidney disease and newly diagnosed arterial hypertension, and we discuss the possible mechanisms and management of hypertension in the course of this rare disease.