This is the first reported case of juvenile SLE presenting with lupus anticoagulant-hypoprothrombinaemia syndrome and diffuse muscular haemorrhage.
Risk of steroid dependency warrants caution in juvenile SLE with hypoprothrombinaemia syndrome; first case leaves optimal long-term therapy open.
Lupus anticoagulants are closely related to systemic lupus erythemathosus (SLE) and to thrombotic events. We describe a 12 year-old girl with a bilateral intramuscular haemorrhage of the gastrocnemius muscles as her main initial presentation of juvenile SLE. Laboratory work-up revealed lupus anticoagulant-hypoprothrombinaemia syndrome (LAHS) with very low levels of factor II due to autoantibodies. She showed a good initial clinical and laboratory response to prednisone therapy, however steroid dependency developed. To the best of our knowledge, this is the first case reported of juvenile SLE presenting with LAHS.
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Yacobovich et al. (2001) studied this question.
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