systemic lupus erythematosus, who developed an aortic arch syndrome with obstruction of the left subclavian artery by thrombus. The left brachial, radial, and ulnar pulses were absent, and the patient complained of numbness of her left hand.We recently encountered a similar patient with a lupus-like illness, with occluded left axillary and subclavian arteries, also giving rise to an aortic arch syndrome. In addition to involvement of a major vessel arising from the aortic arch, she experienced recurrent cerebrovascular accidents (computerized tomography results were positive), had ii false-positive result on VDRL testing, and had antibodies to cardiolipin and demonstrated the presence of the lupus anticoagulant in serum studies.The patient, a 50-year-old white woman, initially presented in 1976, at age 42, with polyarthralgias, weakness, and lethargy. Three years later, in 1979, she developed a right-sided hemiparesis accompanied by hemianesthesia and a right homonymous hemianopia. Several months later, she complained of headaches and diplopia. Laboratory investigations showed a moderately elevated erythrocyte sedimentation rate (39 mmihour), positive antinuclear factor (ANF), and a reduced C3 of 480 mg% (normal >700 mglliter). Over the next 2 years, she developed Raynaud's phenomenon, dry gritty eyes, lapses of memory, and incoordination. Migrainous headaches became more frequent, and polyarthralgias and myalgias were prominent. Systemic steroid therapy (prednisone, 10 mg daily) was begun in mid-1982. By then, she had become hypertensive (2001110 mm Hg), with mitral incompetence, and she was treated with diuretic therapy (furosemide, 40 mg daily).Livedo reticularis and an erythematous rash (not involving the face) were noted. When admitted to the Hammersmith Hospital in 1983, she had absent left brachial and radial pulses, and an arch aortogram showed complete occlusion of the left axillary artery extending from the subclavian artery which showed severe stenosis. There was no evidence of atheromatous disease in any vessels. Computerized tomography showed multiple widespread cerebral infarcts.Laboratory studies showed the following results: negative DNA binding of 8-12% (normal <30%), negative antibodies to extractable nuclear antigens, and negative rheumatoid factor (latex agglutination). The A N F was still weakly positive (1 :40), with a positive Coombs' test, and at this stage, it was noted that the antibodies to cardiolipin were strongly positive. The test for lupus anticoagulant was positive, partial thromboplastin time was 49 seconds (control 15), and she also had a false-positive result on VDRL. The serum cholesterol value averaged 5.9 mmoles/liter over a 9-month period.She was treated with steroids (prednisone, 10 mg daily), regular pulses of cyclophosphamide (500 mg), and plasma exchanges.Although our patient was 52 years of age, her symptoms had begun when she was 42. There was no clinical or radiologic evidence of atherosclerosis in any other major vessels of the arch o r abdominal aorta. Serum chole...