A 48-year-old woman with a history of systemic lupus erythematosus (SLE) presented to the emergency department from the rheumatology clinic for digital ischemia. The clinical manifestations of her SLE consisted predominantly of arthralgias, which had been previously well controlled on hydroxychloroquine 300 mg/d PO. On presentation, she denied oral ulcers, alopecia, shortness of breath, chest pain/pressure, and history of blood clots or miscarriages.
On exam, the patient was afebrile and had a heart rate of 74 bpm; blood pressure, 140/77 mm Hg; and respiratory rate, 18 breaths/min. The fingertips on her left hand were tender and cool to the touch, and the fingertips of her second through fifth digits were blue (FIGURE).
Laboratory workup was notable for the following: hemoglobin, 9.3 g/dL (normal range, 11.6-15.2 g/dL) and erythrocyte sedimentation rate, 44 mm/h (normal range, ≤ 25 mm/h). Double-stranded DNA and complement levels were normal.
Transthoracic echocardiogram did not show any valvular vegetations, and blood cultures from admission were negative. Computed tomography angiography (CTA) with contrast of her left upper extremity showed a filling defect in the origin of the left subclavian artery. Digital plethysmography showed dampened flow signals in the second through fifth digits of the left hand.
Tests for antiphospholipid antibodies were positive for lupus anticoagulant; there were also high titers of anti-β-2-glycoprotein immunoglobulin (Ig) G (58 SGU; normal, ≤ 20 SGU) and anticardiolipin IgG (242.4 CU; normal, ≤ 20 CU).
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