Subarachnoid hemorrhage and dural sinus thrombosis are important manifestations of neuropsychiatric lupus erythematosus. We report the case of a woman with relapsed lupus nephritis, in partial remission, who presented with the unusual combination of dural sinus thrombosis (due to protein S functional deficiency) and aneurysmal subarachnoid hemorrhage. She had a fatal outcome, as has often been reported with subarachnoid hemorrhage in the context of active lupus. To our knowledge, this is the first report of such an unusual association in lupus, and highlights unique challenges in the management of intracranial hemorrhage in the context of dural sinus thrombosis.
BertsiasGKBoumpasDT. Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol2010; 6: 358–367.
2.
MimoriASuzukiTHashimotoM. Subarachnoid hemorrhage and systemic lupus erythematosus. Lupus2000; 9: 521–526.
3.
GinsbergJSDemersCBrill-EdwardsP. Acquired free protein S deficiency is associated with antiphospholipid antibodies and increased thrombin generation in patients with systemic lupus erythematosus. Am J Med1995; 98: 379–383.
SharmaSSharmaNYeolekarME. Acute subarachnoid hemorrhage as initial presentation of dural sinus thrombosis. J Neurosci Rural Pract2010; 1: 23–25.
7.
El OtmaniHMoutaouakilFFadelHSlassiI. [Subarachnoid hemorrhage induced by cerebral venous thrombosis]. J Mal Vasc2012; 37: 323–325.
8.
LaversuchCJBrownMMCliftonABourkeBE. Cerebral venous thrombosis and acquired protein S deficiency: An uncommon cause of headache in systemic lupus erythematosus. Br J Rheumatol1995; 34: 572–575.
9.
GuermaziSRegnaultVGorgiYAyedKLecompteTDellagiK. Further evidence for the presence of anti-protein S autoantibodies in patients with systemic lupus erythematosus. Blood Coagul Fibrinolysis2000; 11: 491–498.
10.
SongKSParkYSKimHK. Prevalence of anti-protein S antibodies in patients with systemic lupus erythematosus. Arthritis Rheum2000; 43: 557–560.
11.
MarlarRAGausmanJN. Laboratory testing issues for protein C, protein S, and antithrombin. Int J Lab Hematol2014; 36: 289–295.
12.
HankeyGJEikelboomJWvan BockxmeerFMLofthouseEStaplesNBakerRI. Inherited thrombophilia in ischemic stroke and its pathogenic subtypes. Stroke2001; 32: 1793–1799.
