Primer Antifosfolipid Sendromu : (Derleme)

Year 2005, Volume: 22 Issue: 2, 100 - 111, 31.12.2009

C. Şahan K. Cengiz

Abstract

Antifosfolipid sendromu (AFS), yükselmiş antifosfolipid antikor titrelerinin eşlik ettiği arteriyel ya da venöz tromboz ve tekrarlayan fetal kayıp kombinasyonu ile karakterize sis-temik otoimmün bir bozukluktur. İki formu tanımlanmıştır: başka hiç bir hastalığın eşlik etmediği "primer sendrom" ve çoğunlukla SLE ile birlikte olan sekonder sendrom. 1998 yılında Sappora'da Uluslararası Uzmanlar Görüş panelinde AFS tanı kriterleri belirlenmiştir. Hasta plazmalarında antifosfolipid antikor varlığını göstermede iki ölçümden yararlanılır; ELİSA ile gösterilen antikardyolipin antikorları ve koagülasyon testleri ile taranan lupus antikoagülanı. Tedavi karar 4 ana grupta verilir: profilaksi, önleme, akut trombozun tedavisi ve antifosfolipid antikor ile birlikte olan gebeliğin tedavisi.



Primary Anti-Phospholipid Syndrome

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a combination of arterial or venous thrombosis and recurrent fetal loss, accompanied by elevated titers of antiphospholipid antibodies (aPL). Two forms have bee described: the "primary syndrome" where there is no evidence of an underlying disease, and the "secondary" syndrome, mainly in the setting of systemic lupus eryhematosus. In 1998; an international panel of experts met in Sapporo to establish the classification criteria for definite antiphos-pholipid syndrome. Two assays are available to demonstrate the presence of antiphospho-lipid antibodies in plasma of patients: ELİSA for the detection of anticardiolipin antibodies and coagulation assays for the detection of the lupus anticoagulants. Treatment decisions fall into four main areas. Prophylaxis, prevention, treatment of acute thrombosis, and management of pregnancy in association with antiphospholipid antibodies.

Keywords

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References

• Levine JS, Branch DW, Rauch J. The antiphospholipid
• syndrome. NEJM 2002; 346: 752-763.
• Greaves M. Antiphospholipid antibodies and thrombosis.
• Lancet 1999; 353: 1348-1353.
• de Groot PG, Derksen RH: Pathophysiology of the
• antiphospholipid syndrome. J Thromb Haemost 2005; 3:1854-1860.
• Conley CL, Hartmann RC. A hemorrhagic disorder
• caused by circulating anticoagulant in patients with
• disseminated lupus erythematosus. J Clin Invest 1952; 31: 621-622.
• Hughes GVR. Thrombosis, abortion, cerebral disease
• and lupus anticoagulant Br Med J 1983; 287: 1088- 1089.
• Hughes GRV, Haris EN, Gharavi AE. The anticardiolipin
• syndrome. J Rheumatol 1986; 13: 486-489
• Piette JC, Wechsler B, Frances C, et al. Exclusion
• c r i t e r i a for primary antiphospholipid syndrome. J
• Rheumatol 1993; 20: 1802-1804.
• Asherson RA. The catastrophic antiphospholipid
• s y n d r o m e . J Rheumatol 1992; 19: 508-512.
• Sneddon IB. Cerebrovascular lesions and livedo
• r e t i c u l a r i s . Br J Dermatol 1965; 77: 180-185.
• Roubey ASR: Autoantibodies to phospholipid-binding plasma proteins: A new view of lupus anticoagulants and other “antiphospholipid” proteins. Blood 1994; 84: 2854-2867.
• Coulam CB, Mc Intyre JA, Wagenknecht D, et al. Inter-laboratory in consistencies in detection of anticardiolipin antibodies. Lancet 1990; 335: 865.
• Peaceman AM, Silver RK, Mac Gregor SN, et al. Interlaboratory variation in antiphospholipid antibody testing. Am J Obstet Gynecol 1992; 166: 1780-1787.
• Wilson WA, Gharavi AE, Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 1999; 42: 1309-1311.
• Gali M, Luciani D, Bertolini G, et al. Lupus anticoagulants are stonger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome. a systematic review of the literature. Blood 2003;101:1827-1832.
• Bick RL. The antiphospholipid thrombosis syndrome : a common multidisciplinary medical problem. J Clin Appl Thromb Heamost 1997; 3: 270-283.
• Bick RL: The antiphospholipid thrombosis syndrome. Hematol Oncol Clin N Am 2003;17:115-147.
• Harris EN, Gharavi AE, Boey ML: Anticardiolipin a n t i b o d i e s . detection by radioimmunoassay and association with thrombosis in SLE. Lancet 1983; 11: 1211-1214.
• Harris EN: The second international anti-cardiolipin standardization workshop/The Kingston antiphospholipid antibody study (KAPS) group. Am J Clin Pathol 1990; 91: 476-484.
• Reber G, de Moerloose P. Anti-beta-2-glycoprotein I antibodies. When and how should they be measured? Thromb Res 2004; 114: 527-531.
• Galli M, Luciani D, Bertolini G, et al. Anti-beta-2- g l y c o p r o t e i n I antibodies, antiprothombin antibodies, and the risk of thrombosis in the antiphospholipid syndrome. Blood 2003; 102: 2717-2723.
• Reber G, Tincani A, Sanmarca M, et al. Proposals for the measurement of anti-beta-2-glycoprotein I antibodies: atandardization group of the European forum on Antiphospholipid Antibodies. J Thromb haemost 2004; 2: 1860-1862.
• de Laat B, Derksen RH, Urbanus RT, et al. Ig G antibodies that recognize epitope Gly 40 – Arg 43 in domain ı of beta-2-glycoproteinI cause LAC and their presence correlates strongly with thrombosis Blood 2005; 105: 1540-1547.
• Cugno M, Cabibbe M, Galli M, et al. Antibodies to tissue- type plasminogen activator (tPA) in patients with antiphospholipid syndrome : evidence of interaction between the antibodies and catalytic domain of tPA in 2 patients. Blood 2004; 103: 2121-2126.
• Yasuda S, Atsumi T, Ieka M, et al. Nicked beta-2- g l y c o p r o t e i n I: a marker of cerebral infarct and a novel role in the negative feedback pathway of e x t r i n s i c fibrinolysis. Blood 2004; 103: 3766-3772.
• Shi T, Iverson GM, Di JC, et al. Beta-2-glycoprotein I bind factor XI and inhibits its activation by thrombin and factor XII a: loss of inhibition by clipped beta-2- glycoprotein I. Proct Natl Acad Sci USA 2004; 101: 3939-3944.
• Shi T, Giannakapoulas B, Iverson GM, et al. Domain V of beta-2-glycoprotein I binds factor XI/XIa and cleaved at Lys317-Thr318. J Biol Chem 2005; 280: 907-912.
• Out HJ, Bruinse HW, Christians GCML, et al. Prevalence of antiphospholipid antibodies in patients with fetal loss. Ann Rheum Dis 1991; 50: 553-557.
• Parazzini F, Acala B, Faden D, et al. Antiphospholipid antibodies and recurrent abortion. Obstet Gynecol 1991; 77: 854-858.
• Yetman DL, Kutteh WH. Antiphospholipid antibody panels and recurrent pregnancy loss: prevalence of anticardiolipin antibodies compared with other antiphospholipid antibodies Fertil Steril 1996; 66: 540-546.
• İnfante-Rivard C, David M, Gauthier R, et al. Lupus anti-coagulants, anticardiolipin antibodies, and fetal loss. A case control study. NEJM 1991; 325: 1063-1066.
• Bociolone L, Meromi P, Parazzini F, et al. Antiphospholipid antibodies and risk of intrauterine late fetal death. Acta Obstet Gynecol Scand 1994; 73: 389-392.
• Branc DW, Silver RM, Blackwell JL, et al. Outcome of treated pregnancies in women with antiphospholipid syndrome : a update of the Utah experience. Obstet Gynecol 1992; 80: 614-620.
• Lima F, Khamashia MA, Buchanan NMN, et al. a study of sixty pregnancies in patients with the antiphospholipid syndrome. Clin Exp Rheumatol 1996; 14: 131-136.
• Yasuda M, Takalcuwa K, Takunago A, et al. Prospective studies of the association between anticardiolipin antibody and outcome of pregnancy. Obstet Gynecol 1995; 86: 555-559.
• Asherson RA, Khamostho MA, ordi-Ros J, et al. The primary antiphosholipid syndrome: a major clinical and serological features. Medicine(Baltimore) 1989; 68: 366-374.
• Alarcon-Segovia D, Perez-Vazguez ME; Villa AR, et al. Preliminary classification criteria for the a n t i p h o s p h o l i p i d syndrome within systemic lupus erythematosus: Semin Arthritis Rheum 1992; 21: 275-286.
• Vianna JL, Khamastha MA, ordi-Ros J, et al. Comparison of the primary and secondary antiphospholipid s y n d r o m e : a European multicenter study of 114 patients. Am J Med 1994; 96: 3-9.
• Malm J, Laurell M, Nilsson IM, et al. Thromboembolic disease critical evaluation of laboratory investigation. Throm Haemost 1992; 68: 7-13
• Silver RM, Draper ML, Scott JR, et al. Clinical consequences of antiphospholipid antibodies: a historic cohort study. Obstet Gynecol 1994; 83: 372-77.
• Italian Registry of antiphospholipid antibodies. Thrombosis and thrombocytopenia in antiphospholipid syndrome. : first report from the Italian Registry. Haematologica 1993; 78: 313-318.
• Asherson RA, Cervera R. Antifosfolipid antibodies and the lung. J Rheumatol 1995; 22: 62-66.
• Weber M, Hayem G, De bandt M, et al. The family history of patients with primary and secondary antiphospholipid syndrome. Lupus 2000; 9: 258-263.
• Koike T, Tsutsumi A. Pulmonary hypertension and the antiphospholipid syndrome. Intern Med 1995; 34: 938-940.
• Cuadrado M, Hughes GRV. Hughes (antiphospholipid) syndrome. Rheum Dis Clin 2001; 27: 507-524.
• Maser KM, Auger WR, Fedolo PF. Chronic major-vessel thromboembolic pulmonary hypertension. Circulation 1990; 81: 1735-1743.
• Jamieson SW, Auger WR, Fedollo PF, et al. Experience and results with 150 pulmonary thromboendartectomy operations over a 29 month period. J Thorac Cardiovasc Surg 1993; 106: 116-127.
• Sandoval J, Amigo ML, Barragon R, et al. Primary antiphospholipid syndrome presenting as chronic thromboembolic hypertension treatment with thromboendartectomy. J Rheumatol 1996; 23: 772-775.
• Hillerdal G. The lung physician and the antiphopholipid syndrome. Eur Respir J. 1997; 10: 511-512.
• Luchi ME, Asherson RA, Lahita RG. Primary idiopathic pulmonary hypertension complicated by pulmonary arterial thrombosis. Association with antiphospholipid antibodies. Arthritis Rheum 1992; 35: 700-705.
• Nagai H, Yosuma K, Katsuki T, et al. Primary antiphospholipid syndrome and pulmonary hypertension with prolonged survival. A case report. Angiology 1997; 48: 183-187.
• de la Mata J, Gomez-Sanchez MA, Aranzone M, et al. Long term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases. Arthritis Rheum 1994; 37: 1528-1532
• Brucato A, Baudo F; Barberis M, et al. Pulmonary hypertension secondary to thrombosis of the p u l m o n a r y vessels in patient with the primary antiphospholipid syndrome. J Rheumatol 1994; 21: 942-944.
• Asherson RA, Cervera R, Piette JC, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine(Baltimore) 1998; 77: 195-207.
• Magiarini M, Knoblauch A, Schneider J, et al. Diffuse microvascular pulmonary thrombosis associated with primary antiphospholipid syndrome. EUr Respir J. 1997; 10: 727-730.
• Gertner E. Diffuse alveolar haemorrhage in the antiphospholipid syndrome: spectrum of disease and treatment J Rheumatol 1999; 26: 805-807.
• Montalban J, Codine A, Ordi J, et al. Antiphopholipid antibodies in cerebral ischemia. Stroke 1991; 22: 750-753.
• Brey RL, Hart RG, Sherman DG, et al. Antiphopholipid antibodies and cerebral ischemia in young people. Neurology 1990; 40: 1190-1196.
• Levine SR, Langer SL, Albers JW. Sneddon syndrome: a antiphopholipid antibody syndrome? Neurology 1988; 38: 798-800.
• Levine SR, Welch K. The spectrum of neurologic dis- ease associated with anticardiolipin antibody. Arch Neurol 1987;44:876-890.
• Tietjen GE, Day M, Narris L, et al. Role of anticardi- olipin antibodies in young persons with migraine and transient focal neurologic events. Neurology 1998; 50: 1433-1440.
• Frances C, Niang S, Laffitte E, et al. Dermatologic manifestations of the antiphospholipid syndrome: two hundred consecutive cases. Arthritis Rheum 2005; 52: 1785-1793.
• Diogenes MJ, Diogenes PC, de Morais Carnerio RM, et al. Cutaneous manifestations associated with antiphospholipid antibodies. Int J Dermatol 2004; 43: 623-627.
• Khamashta MA. Management of thrombosis in the antiphospholipid syndrome. Lupus 1996;5:463-466
• Ginsburg KS, Liang MH, Newcower L, et al. Anticardiolipin antibodies and the risk for ischemic stroke and venous thrombosis. Ann Intern Med 1992;117:997-1002.
• Erkan D, Merrill JT, Yazici Y, et al. High thrombosis rate after fetal loss in antiphospholipid syndrome. Effective prophylaxis with aspirin. Arthritis Rheum 2001; 44: 1466-1467.
• Petri M. Hydroxychloroquine use in Baltimore Lupus Cohort: Effects on lipids, glucose and thrombosis. Lupus 1996; 5(Supp1): 516-522.
• Crowther MA, Ginsberg JS, Julian J, et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. NEJM 2003; 349: 1133-1138.
• Pattison NS, Chamley İW, Birdsall M, et al. Does aspirin have a role in improving pregnancy outcome for women with the antiphospholipid syndrome? A randomized controlled trial. Am J Obstet Gynecol 2000; 183: 1008-1012.
• Branch DW; Khamastha MA: Antiphospholipid s y n d r o m e : Obstetric diagnosis, management and contraversies. Obstet gynecol. 2003; 101: 1333-1344.
• Lockshin MD. Antiphospholipid syndrome. Rheum Dis Clin Of North Am 1994; 20: 45-59.
• Branch DW, Peaceman AM, Druzin M, et al. A multicenter, placebo-controlled pilot study of intravenous immunoglobulin treatment of antiphospholipid s y n d r o m e during pregnancy. Am J Obstet Gynecol. 2000; 182: 122-127.
• Triolo G, Ferrante A, Ciccia F, et al. Randomized study of subcutaneous low molecular weight heparin plus aspirin versus intravenous immunoglobulin in the treatment of recurrent pregnancy loss associated with antiphospholipid antibodies. Arthritis Rheum, 2003; 48: 728-731.
• Asherson RA, Cerves R, Piette JC, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine(Baltimore) 1998; 77: 195-207.