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CMDT 2008
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Pregnancy complications, specifically pregnancy losses after the first trimester Lifelong anticoagulation with warfarin is recommended currently for patients with serious complications of this syndrome, as recurrent events are common
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A primary antiphospholipid antibody syndrome (APS) is diagnosed in patients who have recurrent venous or arterial occlusions, recurrent fetal loss, or thrombocytopenia in the presence of antiphospholipid antibodies but not other features of SLE In less than 1% of patients with antiphospholipid antibodies, a potentially devastating syndrome known as the catastrophic antiphospholipid syndrome occurs, leading to diffuse thromboses, thrombotic microangiopathy, and multiorgan system failure
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A Symptoms and Signs
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Patients are often asymptomatic until suffering a thrombotic complication of this syndrome Thrombotic events may occur in either the arterial or venous circulations Thus, deep venous thromboses, pulmonary emboli, cerebrovascular accidents are typical clinical events among patients with the APS Budd-Chiari syndrome, cerebral sinus vein thrombosis, myocardial or digital infarctions, and other thrombotic events also occur A variety of other symptoms and signs are often attributed to the APS, including mental status changes, livedo reticularis, skin ulcers, microangiopathic nephropathy, and cardiac valvular dysfunction typically mitral regurgitation that may mimic Libman-Sacks endocarditis Livedo reticularis is strongly associated with the subset of patients with APS in whom arterial ischemic events develop
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Appropriate management of SLE requires the active participation of a rheumatologist The severity of organ involvement dictates referral to other subspecialists, such as nephrologists and pulmonologists
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Ginzler EM Mycophenolate mofetil or intravenous cyclophosphamide for lupus nephritis N Engl J Med 2005 Nov 24; 353(21):2219 28 [PMID: 16306519] Petri M et al; OC-SELENA Trial Combined oral contraceptives in women with systemic lupus erythematosus N Engl J Med 2005 Dec 15;353(24):2550 8 [PMID: 16354891] Somers EC et al Use of a gonadotropin-releasing hormone analog for protection against premature ovarian failure during cyclophosphamide therapy in women with severe lupus Arthritis Rheum 2005 Sep;52(9):2761 7 [PMID: 16142702] Tseng CE et al The effect of moderate-dose corticosteroids in preventing severe flares in patients with serologically active, but clinically stable, systemic lupus erythematosus: findings of a prospective, randomized, double-blind, placebo-controlled trial Arthritis Rheum 2006 Nov;54(11):3623 32 [PMID: 17075807]
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B Laboratory Findings
As noted in the discussion of SLE, three types of antiphospholipid antibody are believed to contribute to this syndrome: (1) anti-cardiolipin antibodies; (2) a lupus anticoagulant that prolongs certain coagulation tests (see below); and (3) an antibody associated with a biologic false-positive test for syphilis (see Table 10 11) Anti-cardiolipin antibodies are typically measured with enzyme immunoassays for either IgG or IgM In general, IgG anti-cardiolipin antibodies are believed to be more pathologic than IgM A clue to the presence of a lupus anticoagulant, which may occur in individuals who do not have SLE, may be detected by a prolongation of the partial thromboplastin time (which, paradoxically, is associated with a thrombotic tendency rather than a bleeding risk) A finding more sensitive for a lupus anticoagulant, however, is prolongation of a specialized coagulation assay known as the Russell viper venom time (RVVT) In the presence of a lupus anticoagulant, the RVVT is prolonged and does not correct with mixing studies With the last type of antiphospholipid antibody, the patient has a positive rapid plasma reagin (RPR), but negative specific anti-treponemal assays
ANTIPHOSPHOLIPID ANTIBODY SYNDROME
ESSENTIALS OF DIAGNOSIS
Hypercoagulability, with recurrent thromboses in either the venous or arterial circulation Thrombocytopenia is common
CMDT 2008
The initial phase of RP, mediated by excessive vasoconstriction, consists of well-demarcated digital pallor or cyanosis; the subsequent (recovery) phase of RP, caused by vasodilation, leads to intense hyperemia and rubor Although RP chiefly affects fingers, it can also affect toes and other acral areas such as the nose and ears RP is classified as primary (idiopathic or Raynaud disease) or secondary Nearly one-third of the population reports being sensitive to the cold but does not experience the paroxysms of digital pallor, cyanosis, and erythema characteristic of RP Primary RP occurs in 2 6% of adults, is especially common in young women, and poses more a nuisance than a threat to good health In contrast, secondary RP is less common, is chiefly associated with rheumatic diseases (especially scleroderma), and is frequently severe enough to cause digital ulceration or gangrene
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