Takayasu's arteritis associated with factor V Leiden

Takayasu's arteritis (TA) is a rare, chronic, and idiopathic vasculitis of the aorta and/or its main branches. There have been case reports of this di sease associated with immune hypercoaguable states, namely raised antiphosp holipid antibodies. Investigations of the thrombotic nature of Takayasu's a rteritis have shown elevated levels of B-thromboglobulin, platelet factor 4 , thrombin-antithrombin III complex, and fibrinopeptide A. We report the fi rst case of TA associated with the Factor V Leiden gene defect (Activated P rotein C Resistance). The patient is a 30-year-old female who presented wit h six months of bilateral lower and upper extremity claudication, carotid a rtery tenderness, diminished brachial pulse and no measurable blood pressur e in the left arm, an erythrocyte sedimentation rate (Westergren) of 62 mm/ hr, and an angiogram meeting the clinical criteria for TA. Her symptoms sho wed a dramatic response to high-dose oral glucocorticosteroids and she was also maintained on long-term anticoagulation, This case illustrates that he reditary hypercoagulable states can coexist with acquired vasculitidies and that further investigation into these associations and their pathophysiolo gic interaction is warranted. Am. 4. Hematol. 60:237-238, 1999, (C) 1999 Wi ley-Liss, Inc.