Central nervous system (CNS) involvement has been recognized as the second leading cause of death in patients with systemic lupus erythematosus (SLE), although hydrocephalus is seen very rarely. We report a case of communicating hydrocephalus in a 24-year-old woman having previously diagnosed SLE without antiphospholipid antibody syndrome or cerebral venous angiographic abnormality. We propose direct damage-thrombosis of small sized venous structures or immune complex deposition within arachnoid villi, which impair cerebrospinal fluid flow, as a possible, yet unproven, pathophysiologic mechanism for hydrocephalus in SLE.

Central nervous system (CNS) involvement has been recognized as the second leading cause of death in patients with systemic lupus erythematosus (SLE), although hydrocephalus is seen very rarely. We report a case of communicating hydrocephalus in a 24-year-old woman having previously diagnosed SLE without antiphospholipid antibody syndrome or cerebral venous angiographic abnormality. We propose direct damage-thrombosis of small sized venous structures or immune complex deposition within arachnoid villi, which impair cerebrospinal fluid flow, as a possible, yet unproven, pathophysiologic mechanism for hydrocephalus in SLE.