Systemic lupus erythematosus with intracerebral hematoma due to decreased factor XIII activity: A rare association

Abstract

Intracerebral hemorrhage is a dangerous occurrence in systemic lupus erythematosus (SLE). This may occur due to hypertension, anticoagulation, aneurysmal rupture, and catastrophic anti-phospholipid antibody syndrome. Factor XIII is required for stabilizing the fibrin clot. Decreased activity of factor XIII is a relatively rare coagulation abnormality, which may be due to congenital and acquired causes. Acquired causes of this condition are drugs such as isoniazid and phenytoin, liver disease, lymphoproliferative disorders, disseminated intravascular coagulation, sepsis and autoimmune disease, including SLE. The association of intra-cerebral hematoma in SLE due to decreased activity of factor XIII as its only detectable cause is a rarity in the medical literature. We report a case of SLE with intra-cerebral hemorrhage presenting as headache, found to be due to decreased activity of factor XIII. The activity of factor XIII increased after treatment with intravenous cyclophosphamide and there were no further bleeds reported.