Pediatric Systemic Lupus Erythematosus
SLE is often associated with a prolonged partial thromboplastin time (PTT) because of the presence of a lupus anticoagulant (Chapter 16, Antiphospholipid Syndrome). However, in approximately 5% of lupus patients, there is also a prolonged prothrombin time (PT). The implications of a prolonged PT in lupus are altogether different. A prolonged PT in lupus is generally the result of an acquired deficiency of factor II or an acquired inhibitor to factor II. Patients with these defects generally present with hemorrhage rather than thrombosis, the hematological event normally linked with lupus anticoagulants. The treatment approach to an acquired factor II deficiency or inhibitor is immunosuppression. Prednisone is the only medication required in most circumstances, and high doses of this medication (e.g., 1–2 mg/kg/day) usually lead to a rapid normalization of the PT (Eberhard et al. 1992; Hudson et al. 1997; Massengill et al. 1997; Taddio et al. 2007).
KeywordsRheumatic Fever Lupus Anticoagulant Macrophage Activation Syndrome Congenital Heart Block Prolonged Prothrombin Time
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