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Antiphospholipid Antibody: Title and subTitle BreakBabies, Blood Clots, Biology FREE

Michael D. Lockshin, MD
[+] Author Affiliations

Corresponding author: Michael D. Lockshin, MD, Barbara Volcker Center for Women and Rheumatic Disease, The Hospital for Special Surgery-Cornell Medical Center, 535 E 70th St, New York, NY 10021 (e-mail: LockshinM@hss.edu).

Grand Rounds at the Clinical Center of the National Institutes of Health section editors: John I. Gallin, MD, the Clinical Center of the National Institutes of Health, Bethesda, Md; David S. Cooper, MD, Contributing Editor, JAMA.


JAMA. 1997;277(19):1549-1551. doi:10.1001/jama.1997.03540430061034
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CASE PRESENTATION  At the age of 22 years, a Hispanic woman developed joint pain, fatigue, rash, and thrombocytopenia. Results of antinuclear and anti-DNA antibody tests were positive. Urinalysis and renal function test results were normal. Systemic lupus erythematosus (SLE) was diagnosed, and hydroxychloroquine sulfate and nonsteroidal anti-inflammatory drugs were prescribed. She did well until she was seen again at age 29 years, when she developed scotomata following a first-trimester spontaneous abortion. Findings of a neurological evaluation, including magnetic resonance imaging, electroencephalography, and Doppler studies, were unremarkable. At age 30 years, severe intrauterine growth restriction, hypertension, and proteinuria complicated a pregnancy, which ended with fetal death at 22 weeks.At age 32 years, the patient sought another opinion. This examination revealed normal vital signs, slight patches of malar erythema, lupus rash, livedo reticularis (Figure), and a loud apical systolic murmur. Her platelet count was 96X109/L. Prothrombin time was 13.0

REFERENCES

Branch DW, Silver RM.  Criteria for antiphospholipid syndrome: early pregnancy loss, fetal loss, or recurrent pregnancy loss? Lupus . 1996;;5:409-413.
Asherson RA, Cervera R, Piette J-C, Shoenfeld Y.  The antiphospholipid syndrome: history, definition, classification, and differential diagnosis . In: Asherson RA, Cervera R, Piette J-C, Shoenfeld Y, eds. The Antiphospholipid Syndrome . Boca Raton, Fla: CRC Press; 1996;:3-12.
Wilson WA.  Histocompatibility genes in the antiphospholipid antibody syndrome . Lupus . 1996;;5: 295-262.
Ingram SB, Goodnight SH Jr, Bennett RM.  An unusual syndrome of a devastating noninflammatory vasculopathy associated with anticardiolipin antibodies: report of two cases . Arthritis Rheum . 1987;;30:1167-1172.
Asherson RA, Piette J-C.  The catastrophic antiphospholipid syndrome 1996: acute multi-organ failure associated with antiphospholipid antibodies: a review of 31 patients . Lupus . 1996;;5:414-417.
Lockshin MD, Druzin ML, Qamar T.  Prednisone does not prevent fetal death in women with antiphospholipid antibody . Am J Obstet Gynecol . 1989;; 160:439-443.
Silver RM, Porter TF, van Leeuween I, et al.  Anticardiolipin antibodies: clinical consequences of low titers . Obstet Gynecol . 1996;;87:494-500.
Stephenson MD.  Frequency of factors associated with habitual abortion in 197 couples . Fertil Steril . 1996;;66:24-29.
Lynch A, Marlar R, Murphy J, et al.  Antiphospholipid antibodies in predicting adverse pregnancy outcome: a prospective study . Ann Intern Med . 1994;;120:470-475.
Silver RM, Draper ML, Scott JR, Lyon JL, Reading J, Branch DW.  Clinical consequences of antiphospholipid antibodies: an historic cohort study . Obstet Gynecol . 1994;;83:372-377.
Khamashta MA, Cuadrado MJ, Mujic F, Taub NA, Hunt BJ, Hughes GRV.  The management of thrombosis in the antiphospholipid-antibody syndrome . N Engl J Med . 1995;;332:993-997.
Ginsburg KS, Liang MH, Newcomer L, et al.  Anticardiolipin antibodies and risk for ischemic stroke and venous thrombosis . Ann Intern Med . 1992;;117:997-1002.
Finazzi G, Brancaccio V, Moia M, et al.  Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: a four-year prospective study from the Italian Registry . Am J Med . 1996;;100:530-536.
Mantaruli B, Borchiellini A, Tamponi G, et al.  Factor V Arg506→ Gln mutation in patients with antiphospholipid antibodies . Lupus . 1996;;5:303-306.
Harris EN, Pierangeli SS.  Functional effects of anticardiolipin antibodies . Lupus . 1996;;5:372-377.
Kandiah DA, Sheng YH, Krilis SA.  β2-glycoprotein I: target antigen for autoantibodies in the 'antiphospholipid syndrome.' Lupus . 1996;;381-385.
Wang M-X, Kandiah DA, Ichikawa K, et al.  Epitope specificity of monoclonal anti-β2-glycoprotein I antibodies derived from patients with the antiphospholipid syndrome . J Immunol . 1995;;155: 1629-1636.
Borchman D, Harris EN, Pierangeli SS, Lamba OP.  Interactions and molecular structure of cardiolipin and β2-glycoprotein 1 (β2-GP1) . Clin Exp Immunol . 1995;;102:373-378.
Mori T, Takeya H, Nishioka J, Gabazza EC, Suzuki K.  Beta(2)-glycoprotein I modulates the anticoagulant activity of activated protein C on the phospholipid surface . Thromb Haemost . 1996;;75: 49-55.
Fishman P, Falach-Vaknine E, Zigelman R, et al.  Prevention of fetal loss in experimental antiphospholipid syndrome by in vivo administration of recombinant interleukin-3 . J Clin Invest . 1993;;91: 1834-1837.
Simantov R, LaSala JM, Lo SK, et al.  Activation of cultured vascular endothelial cells by antiphospholipid antibodies . J Clin Invest . 1995;;96:2211-2219.
Olee T, Pierangeli SS, Handley HH, et al.  A monoclonal IgG anticardiolipin antibody from a patient with the antiphospholipid syndrome is thrombogenic in mice . Proc Natl Acad Sci USA . 1996;; 93:8606-8611.
Roubey RAS.  Immunology of the antiphospholipid antibody syndrome . Arthritis Rheum . 1996;; 39:1444-1454.
Casciola-Rosen L, Rosen A, Petri M, Schlissel M.  Surface blebs on apoptotic cells are sites of enhanced procoagulant activity: implications for coagulation events and antigenic spread in systemic lupus erythematosus . Proc Natl Acad Sci USA . 1996;;93:1624-1629.
Kutteh WH.  Antiphospholipid antibody-associated recurrent pregnancy loss: treatment with heparin and low-dose aspirin is superior to lowdose aspirin alone . Am J Obstet Gynecol . 1996;;174: 1584-1589.
Douketis JD, Ginsberg JS, Burrows RF, Duku EK, Webber CE, Brill-Edwards P.  The effects of long-term heparin therapy during pregnancy on bone density: a prospective matched cohort study . Thromb Haemost . 1996;;75:254-257.
Ginsberg JS, Wells PS, Brill-Edwards P, et al.  Antiphospholipid antibodies and venous thromboembolism . Blood . 1995;;86:3685-3691.
Moll S, Ortel TL.  The INR is invalid in monitoring warfarin anticoagulant therapy in patients with lupus anticoagulants . Lupus . 1996;;5:522. Abstract.
Brey RL, Levine SR.  Treatment of neurologic complications of antiphospholipid antibody syndrome . Lupus . 1996;;5:473-476.
Cowchock FS, Reece EA, Balaban D, Branch DW, Plouffe L.  Repeated fetal losses associated with antiphospholipid antibodies: a collaborative randomized trial comparing prednisone to low-dose heparin treatment . Am J Obstet Gynecol . 1992;;166: 1318-1323.
Pregnancy Loss Study Group.  Randomized, placebo-controlled trial of intravenous immune globulin (IVIG) in antiphospholipid syndrome (APS) in pregnancy: a progress report . Lupus . 1996;;5:201. Abstract.

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Branch DW, Silver RM.  Criteria for antiphospholipid syndrome: early pregnancy loss, fetal loss, or recurrent pregnancy loss? Lupus . 1996;;5:409-413.
Asherson RA, Cervera R, Piette J-C, Shoenfeld Y.  The antiphospholipid syndrome: history, definition, classification, and differential diagnosis . In: Asherson RA, Cervera R, Piette J-C, Shoenfeld Y, eds. The Antiphospholipid Syndrome . Boca Raton, Fla: CRC Press; 1996;:3-12.
Wilson WA.  Histocompatibility genes in the antiphospholipid antibody syndrome . Lupus . 1996;;5: 295-262.
Ingram SB, Goodnight SH Jr, Bennett RM.  An unusual syndrome of a devastating noninflammatory vasculopathy associated with anticardiolipin antibodies: report of two cases . Arthritis Rheum . 1987;;30:1167-1172.
Asherson RA, Piette J-C.  The catastrophic antiphospholipid syndrome 1996: acute multi-organ failure associated with antiphospholipid antibodies: a review of 31 patients . Lupus . 1996;;5:414-417.
Lockshin MD, Druzin ML, Qamar T.  Prednisone does not prevent fetal death in women with antiphospholipid antibody . Am J Obstet Gynecol . 1989;; 160:439-443.
Silver RM, Porter TF, van Leeuween I, et al.  Anticardiolipin antibodies: clinical consequences of low titers . Obstet Gynecol . 1996;;87:494-500.
Stephenson MD.  Frequency of factors associated with habitual abortion in 197 couples . Fertil Steril . 1996;;66:24-29.
Lynch A, Marlar R, Murphy J, et al.  Antiphospholipid antibodies in predicting adverse pregnancy outcome: a prospective study . Ann Intern Med . 1994;;120:470-475.
Silver RM, Draper ML, Scott JR, Lyon JL, Reading J, Branch DW.  Clinical consequences of antiphospholipid antibodies: an historic cohort study . Obstet Gynecol . 1994;;83:372-377.
Khamashta MA, Cuadrado MJ, Mujic F, Taub NA, Hunt BJ, Hughes GRV.  The management of thrombosis in the antiphospholipid-antibody syndrome . N Engl J Med . 1995;;332:993-997.
Ginsburg KS, Liang MH, Newcomer L, et al.  Anticardiolipin antibodies and risk for ischemic stroke and venous thrombosis . Ann Intern Med . 1992;;117:997-1002.
Finazzi G, Brancaccio V, Moia M, et al.  Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: a four-year prospective study from the Italian Registry . Am J Med . 1996;;100:530-536.
Mantaruli B, Borchiellini A, Tamponi G, et al.  Factor V Arg506→ Gln mutation in patients with antiphospholipid antibodies . Lupus . 1996;;5:303-306.
Harris EN, Pierangeli SS.  Functional effects of anticardiolipin antibodies . Lupus . 1996;;5:372-377.
Kandiah DA, Sheng YH, Krilis SA.  β2-glycoprotein I: target antigen for autoantibodies in the 'antiphospholipid syndrome.' Lupus . 1996;;381-385.
Wang M-X, Kandiah DA, Ichikawa K, et al.  Epitope specificity of monoclonal anti-β2-glycoprotein I antibodies derived from patients with the antiphospholipid syndrome . J Immunol . 1995;;155: 1629-1636.
Borchman D, Harris EN, Pierangeli SS, Lamba OP.  Interactions and molecular structure of cardiolipin and β2-glycoprotein 1 (β2-GP1) . Clin Exp Immunol . 1995;;102:373-378.
Mori T, Takeya H, Nishioka J, Gabazza EC, Suzuki K.  Beta(2)-glycoprotein I modulates the anticoagulant activity of activated protein C on the phospholipid surface . Thromb Haemost . 1996;;75: 49-55.
Fishman P, Falach-Vaknine E, Zigelman R, et al.  Prevention of fetal loss in experimental antiphospholipid syndrome by in vivo administration of recombinant interleukin-3 . J Clin Invest . 1993;;91: 1834-1837.
Simantov R, LaSala JM, Lo SK, et al.  Activation of cultured vascular endothelial cells by antiphospholipid antibodies . J Clin Invest . 1995;;96:2211-2219.
Olee T, Pierangeli SS, Handley HH, et al.  A monoclonal IgG anticardiolipin antibody from a patient with the antiphospholipid syndrome is thrombogenic in mice . Proc Natl Acad Sci USA . 1996;; 93:8606-8611.
Roubey RAS.  Immunology of the antiphospholipid antibody syndrome . Arthritis Rheum . 1996;; 39:1444-1454.
Casciola-Rosen L, Rosen A, Petri M, Schlissel M.  Surface blebs on apoptotic cells are sites of enhanced procoagulant activity: implications for coagulation events and antigenic spread in systemic lupus erythematosus . Proc Natl Acad Sci USA . 1996;;93:1624-1629.
Kutteh WH.  Antiphospholipid antibody-associated recurrent pregnancy loss: treatment with heparin and low-dose aspirin is superior to lowdose aspirin alone . Am J Obstet Gynecol . 1996;;174: 1584-1589.
Douketis JD, Ginsberg JS, Burrows RF, Duku EK, Webber CE, Brill-Edwards P.  The effects of long-term heparin therapy during pregnancy on bone density: a prospective matched cohort study . Thromb Haemost . 1996;;75:254-257.
Ginsberg JS, Wells PS, Brill-Edwards P, et al.  Antiphospholipid antibodies and venous thromboembolism . Blood . 1995;;86:3685-3691.
Moll S, Ortel TL.  The INR is invalid in monitoring warfarin anticoagulant therapy in patients with lupus anticoagulants . Lupus . 1996;;5:522. Abstract.
Brey RL, Levine SR.  Treatment of neurologic complications of antiphospholipid antibody syndrome . Lupus . 1996;;5:473-476.
Cowchock FS, Reece EA, Balaban D, Branch DW, Plouffe L.  Repeated fetal losses associated with antiphospholipid antibodies: a collaborative randomized trial comparing prednisone to low-dose heparin treatment . Am J Obstet Gynecol . 1992;;166: 1318-1323.
Pregnancy Loss Study Group.  Randomized, placebo-controlled trial of intravenous immune globulin (IVIG) in antiphospholipid syndrome (APS) in pregnancy: a progress report . Lupus . 1996;;5:201. Abstract.
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