Case of a 9-year-old girl who developed multiple thrombi in brain sinus during oral prednisolone therapy against SLE

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  • 全身性エリテマトーデス治療中に多発性脳静脈血栓を発症した女児例

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Abstract

A 9-year-old girl was admitted to our hospital because of fever, facial erythema and lacy exanthema of her upper and lower limbs. On the basis of clinical and laboratory findings, she was diagnosed as having systemic lupus erythematodes (SLE) and was given oral prednisolone (PSL) therapy. After one month of hospitalization, however, she complained of severe headache in the left occipital region, and multiple thrombi in the superior sagittal venous sinus and sigmoid sinus were detected on magnetic resonance imaging. Neither coagulation nor anticoagulation factors showed any evidence of abnormalities. She was also negative for the lupus anticoagulant and an anti-cardiolipin antibody. She was positive only for anti-phosphatidylserine-prothrombin (aPS/PT) complex antibodies in her plasma. This suggests that her cerebral thrombosis was due to antiphospholipid syndrome (APS) associated with SLE. PSL might also be involved in the generation of thrombi in her brain. Here, we discuss these possibilities as well as the importance of the aPS/PT test as a new tool for diagnosing APS.

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