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Ocular involvement in primary antiphospholipid syndrome

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Abstract

The purpose of this study is to evaluate the ocular findings in patients with the primary antiphospholipid syndrome (APS). Patients and methods: Twenty-two patients (44 eyes) with primary APS (17 women, 5 men) were examined. All patients were younger than 50 years (median age; 37.5 years). In 18 patients, fundus flourescein angiography was performed in addition to the ophthalmologic examination. Results: Sixteen patients (72.7%) described visual symptoms. Anterior segment was normal in 19 patients (86.4%). Posterior segment abnormalities were observed in 15 patients (68.2%). Venous dilatation and tortuosity were the most common ocular findings. Retinal vascular occlusive disease was detected in 5 patients (22.7%). Flourescein angiography abnormalities were noted in 14 of the 18 patients (77.8%). The most common angiographic finding was pigment epithelial window defects. Conclusions: Our results indicate that posterior eye segment involvement is relatively common in the primary APS. It also seems that the screening for APS is important in young patients with retinal vascular occlusion, especially in those without conventional risk factors.

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References

  1. Harris EN. Antiphospholipid antibodies. Br J Haematol 1990; 74: 1–9.

    PubMed  CAS  Google Scholar 

  2. Levine SR, Crofts JW, Lesser GR, Floberg J, Welch KMA. Visual symptoms associated with the presence of a lupus anticoagulant. Ophthalmology 1988; 95: 686–692.

    PubMed  CAS  Google Scholar 

  3. Snyers B, Lambert M, Hardy JP. Retinal and choroidal vasoocclusive disease in systemic lupus erythematosus associated with antiphospholipid antibodies. Retina 1990; 10: 255–260.

    PubMed  CAS  Google Scholar 

  4. Castañón C, Amigo MC, Banales JL, Nava A, Reyes PA. Ocular vaso−occlusive disease in primary antiphospholipid syndrome. Ophthalmology 1995: 102: 256–262.

    PubMed  Google Scholar 

  5. Jonas J, Kölble K, Völcker HE, Kalden JR. Central retinal artery occlusion in Sneddon’s disease associated with antiphospholipid antibodies. Am J Ophthalmol 1986; 102: 37–40.

    PubMed  CAS  Google Scholar 

  6. Kleiner RC, Najarian LV, Schatten S, Jabs DA, Patz A, Kaplan HJ. Vaso−occlusive retinopathy associated with antiphospholipid antibodies (lupus anticoagulant retinopathy). Ophthalmology 1989; 96: 896–904.

    PubMed  CAS  Google Scholar 

  7. Watts MT, Greaves M, Rennie IG, Clearkin LG. Antiphospholipid antibodies in the aetiology of ischemic optic neuropathy. Eye 1991; 5: 75–79.

    PubMed  Google Scholar 

  8. Dori D, Gelfand YA, Brenner B, Miller B. Cilioretinal artery occlusion: an ocular complication of primary antiphospholipid syndrome. Retina 1997; 17: 555–557.

    PubMed  CAS  Google Scholar 

  9. Harris N, Pierangeli S. The Anticardiolipin ELISA. Techniques in Diagnostic Pathology 1991; 2: 21–42.

    CAS  Google Scholar 

  10. Triplett DA, Brandt JT, Kaczor D, Schaeffer J. Laboratory diagnosis of lupus inhibitors: A comparison of the tissue thromboplastin inhibition procedure with a new platelet neutralization procedure. Am J Clin Pathol 1983; 79: 679–682.

    Google Scholar 

  11. Ridker PM, Hennekens CH, Lindpaintner K, Stampfer MJ, Eisenberg PR, Miletich JP. Mutation in the gene coding for coagulation factor V and the risk of myocardial infarction, stroke, and venous thrombosis in apparently healthy men. N Engl J Med 1994; 332: 912–917.

    Article  Google Scholar 

  12. Svensson PJ, Dahlbäck B. Resistance to activated protein C as a basis for venous thrombosis. N Engl J Med 1994; 330: 517–522.

    Article  PubMed  CAS  Google Scholar 

  13. Dhôte R, Bachmeyer C, Horellou MH, Toulon P, Christoforov B. Central retinal vein thrombosis associated with resistance to activated protein C. Am J Ophthalmol 1995; 120: 388–389.

    PubMed  Google Scholar 

  14. Bertina RM, Koeleman BPC, Koster T, Rosendaal FR, Dirven RJ, de Ronde H, et al. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature 1994; 369: 64–67.

    Article  PubMed  CAS  Google Scholar 

  15. Vine AK, Samama MM. The role of abnormalities in the anticoagulant and fibrinolytic systems in retinal vascular occlusions. Surv Ophthalmol 1993; 37: 283–292.

    Article  PubMed  CAS  Google Scholar 

  16. Ordi−Ros J, Perez−Peman P, Monasterio J. Clinical and therapeutic aspects associated to phospholipid binding antibodies (lupus anticoagulant and anticardiolipin antibodies). Haemostasis 1994; 24: 165–174.

    PubMed  CAS  Google Scholar 

  17. McNeil HP, Chesterman CN, Krilis SA. Anticardiolipin antibodies and lupus anticoagulants comprise separate antibody subgroups with different phospholipid binding characteristics. Br J Haematol 1989; 73: 506–513.

    PubMed  CAS  Google Scholar 

  18. Espinoza LR, Hartmann RC. Significance of the lupus anticoagulant. Am J Hematol 1986; 22: 331–337.

    PubMed  CAS  Google Scholar 

  19. Oosting JD, Derksen RH, Bobbink IW, Hackeng TM, Bouma BN, de Groot PG. Antiphospholipid antibodies directed against a combination of phospholipids with prothrombin, protein C, or protein S: an explanation for their pathogenic mechanism? Blood 1993; 81: 2618–2625.

    PubMed  CAS  Google Scholar 

  20. Rand JH, Wu X−X, Andree HA, Ross JB, Rusinova F, Gascon−Lema MG, et al. Antiphospholipid antibodies accelerate plasma coagulation by inhibiting annexin−V biding to phospholipids: A “Lupus Procoagulant” phenomenon. Blood 1998; 92: 1652–1660.

    PubMed  CAS  Google Scholar 

  21. Rand PH. Antiphospholipid antibody syndrome: new insights on thrombogenic mechanisms. Am J Med Sci 1998; 316: 142–151.

    Article  PubMed  CAS  Google Scholar 

  22. Boets EP, Chaar CG, Ronday K, Keunen JE, Breedveld FC. Chorioretinopathy in primary antiphospholipid syndrome: a case report. Retina 1998; 18: 382–385.

    PubMed  CAS  Google Scholar 

  23. Wiechens B, Schröder JO, Pötzsch B, Rochels R. Primary antiphospholipid antibody syndrome and retinal occlusive vasculopathy. Am J Ophthalmol 1997; 123: 848–850.

    PubMed  CAS  Google Scholar 

  24. Pulido JS, Ward LM, Fischman GA, Goodwin JA, Froelich CJ, Sanghvi JP. Antiphospholipid antibodies associated with retinal vascular disease. Retina 1987; 7: 215–218.

    PubMed  CAS  Google Scholar 

  25. Asherson RA, Merry P, Acheson JF, Harris EN, Hughes CR. Antiphospholipid antibodies: a risk factor for occlusive ocular vascular disease in systemic lupus erythematosus and the the ‘primary’ antiphospholipid syndrome. Ann Rheum Dis 1989; 48: 358–361

    PubMed  CAS  Google Scholar 

  26. Acheson JF, Gregson RMC, Merry P, Schulenburg WE. Vasoocclusive retinopathy in the primary antiphospholipid antibody syndrome. Eye 1991; 5: 48–55.

    PubMed  Google Scholar 

  27. Asherson RA, Cervera R. The Antiphospholipid Syndrome: a syndrome in evaluation. Ann Rheum Dis 1992; 51: 147–150.

    Article  PubMed  CAS  Google Scholar 

  28. Ginsburg KS, Liang MH, Newcomer L, Goldhaber SZ, Schur PH, Hennekens CH, et al. Anticardiolipin antibodies and the risk for ischemic stroke and venous thrombosis. Ann Intern Med 1992; 117: 997–1002.

    PubMed  CAS  Google Scholar 

  29. Amigo MC, Garcia−Torres R, Robbes M, Bochicchio T, Reyes PA. Renal involvement in primary antiphospholipid syndrome. J Rheumatol 1992; 19: 1181–1185.

    PubMed  CAS  Google Scholar 

  30. Glacet−Bernard A, Bayani N, Chretien P, Cochard C, Lelong F, Coscas G. Antiphospholipid antibodies in retinal vascular occlusions. Arch Ophthalmol 1994; 112: 790–795.

    PubMed  CAS  Google Scholar 

  31. Coniglio M, Platania A, Di Nucci GD, Arcieri P, Modzrewska R, Mariani G. Antiphosholipid−protein antibodies are not an uncommon feature in retinal venous occlusions. Thromb Res 1996; 83: 183–188.

    Article  PubMed  CAS  Google Scholar 

  32. Brenner B, Vulfsons SL, Lanir N, Nahir M. Coexistence of familial antiphospholipid syndrome and factor V Leiden: Impact on thrombotic diathesis. Br J Haematol 1996; 94: 166–167.

    Article  PubMed  CAS  Google Scholar 

  33. Montaruli B, Borchiellini A, Tamponi C, Giorda L, Bessone P, van Mourik JA, et al. Factor V Arg506→Gln mutation in patients with antiphospholipid antibodies. Lupus 1996; 5: 303–306.

    PubMed  CAS  Google Scholar 

  34. Picillo U, De Lucia D, Palatiello E, Scuotto A, Marcialis MR, Pezzella S, et al. Association of primary antiphospholipid syndrome with inherited activated protein C resistance. J Rheumatol 1998; 25: 1232–1234.

    PubMed  CAS  Google Scholar 

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Demirci, F.Y.K., Küçükkaya, R., Akarçay, K. et al. Ocular involvement in primary antiphospholipid syndrome. Int Ophthalmol 22, 323–329 (1998). https://doi.org/10.1023/A:1006305705080

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