Ocular manifestations of primary thrombotic microangiopathy

Abstract

The article reviews literature on ocular manifestations of primary thrombotic microangiopathy. Thrombotic microangiopathy is a clinical and morphological syndrome that characterizes a systemic disseminated lesion of the microcirculatory bed, it is a special type of vascular damage that combines thrombosis and inflammation of the vascular wall leading to occlusion of the vessel lumen, causing ischemic damage to various organs and tissues. The classic types of primary thrombotic microangiopathy are thrombotic thrombocytopenic purpura, shigatoxin-associated hemolytic uremic syndrome (STEC-HUS) and atypical hemolytic uremic syndrome. Thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome are characterized by ischemic damage to the visual organ as a result of suspected thrombotic microangiopathy in the microcirculatory bed of the eye. The clinical picture of ocular manifestations of these diseases is similar, however the damage to the eye in the form of ischemic Purtscher-like retinopathy is more characteristic of atypical hemolytic uremic syndrome, which may be due to chronic uncontrolled activation of the alternative complement pathway in this disorder. Timely initiation of systemic therapy of the underlying disease in most cases leads to complete restoration of visual functions. A thorough ophthalmological examination of patients with these diseases is required.