Aortic arch syndrome associated with anticadiolipin antibodies and the lupus anticoagulant: Comment on Ferrante paper

Abstract

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...

Antiphospholipid-Thrombosis Syndromes

“…Although there are similarities, there are, at times, clinical, laboratory, and biochemical differences, particularly regarding prevalence, etiology, possible mechanisms of thrombosis, clinical presentations, diagnosis, and at times, management [4,5]. The ACLA-thrombosis antiphospholipid syndrome is much more common than the LA-thrombosis antiphospholipid syndrome, the ratio being approximately 5 to 1 [3,6,7]. All of these syndromes may be associated with (1) arterial and venous thrombosis, (2) recurrent miscarriage, and (3) thrombocytopenia, in descending order of prevalence; however, the anticardiolipin syndrome is associated more commonly with both arterial and venous thrombosis, including typical deep vein thrombosis and pulmonary embolus, premature coronary artery disease, premature cerebrovascular disease (including TIAs, small-stroke syndrome, and cerebrovascular thrombotic stroke), and retinal arterial and venous occlusive disease.…”

“…Antiphospholipid syndrome is the most common acquired blood protein defect associated with either venous or arterial thrombosis or both [2]. The thrombotic and thrombo-occlusive events associated with these antiphospholipid antibodies include thrombosis of the venous system, the arterial system, coronary artery thrombosis, cerebrovascular thrombosis, transient cerebral ischemic attacks (TIAs), retinal vascular thrombosis, and placental vascular thrombosis (leading to recurrent-miscarriage syndrome); these antibodies also may be associated with related clinical syndromes, as discussed in this article [3].…”

“…The types of antiphospholipid and thrombosis syndromes associated with ACLAs are summarized in Box 1 [3,5,69]. Although there seems to be no correlation with the type or titer of ACLA and the type of syndrome (I -VI), the subclassification of thrombosis and ACLA patients into these groups is important from the therapy standpoint [3,5,69]. Type I patients are managed best by use of long-term, fixed-dose LMWH or fixed-dose subcutaneous unfractionated heparin therapy.…”

“…Clopidogrel (at 75 mg/day) is usually effective for retinal vascular thrombosis, and if failure occurs, LMWH is added to the clopidogrel therapy. Therapy for type IV syndrome depends on the types and sites of thrombosis present [3,5,69]. Patients with type V, RMS, are treated best with preconception initiation of low-dose aspirin (81 mg/day) as soon as the diagnosis is made and then started on fixed low-dose porcine mucosal heparin (5000 U subcutaneously every 12 hours) immediately after conception, with both drugs being used to term delivery.…”

“…Obviously, patients with thrombosis and ACLAs require long-term antithrombotic therapy, and treatment should be stopped only if the ACLA is persistently absent for at least 6 months before considering cessation of antithrombotic therapy [3,5,69]. After persistent absence of the antiphospholipid antibody for at least 6 months, the author usually discusses the risks and benefits of continuing antithrombotic therapy and encourages patients to take one lowdose aspirin (81 mg/day) or long-term clopidogrel (depending on the seriousness of the initial thrombotic event, in the hopes that the antibody and thrombosis will not return).…”