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Hughes syndrome (the antiphospholipid syndrome): 25 years old

  • Review Article
  • Published:
Modern Rheumatology

Abstract

The antiphospholipid (Hughes) syndrome (APS) is a unique thrombotic disorder, causing both arterial and venous thrombosis, linked to the presence of antibodies directed against phospholipid–protein complexes. The first papers describing the syndrome were published in 1983 and, over the next two years, a series of publications described in detail the various clinical manifestations of the syndrome. Laboratory standardisation workshops were also set up and, in 1984, the first “world” symposium on APS was held. The international APS conferences have continued to grow in numbers and in stature. The APS has already had an impact in obstetrics, in medicine, in psychiatry, and in surgery. The approximate figure of 1 in 5 is a useful guide—1 in 5 of all young strokes, 1 in 5 recurrent miscarriages, 1 in 5 DVTs. More precise data will become available in the worlds of epilepsy, migraine, Alzheimer’s, and MS. The advent of newer “biologic” immunosuppressives such as rituximab may offer help in selected cases. Intravenous immunoglobulin has proved successful, especially in the emergency setting.

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References

  1. Hughes GR. Thrombosis, abortion, cerebral disease, and the lupus anticoagulant. Br Med J (Clin Res Ed). 1983;287(6399):1088–9.

    CAS  Google Scholar 

  2. Harris EN, Gharavi AE, Patel SP, Hughes GR. Evaluation of the anti-cardiolipin antibody test: report of an international workshop held 4 April 1986. Clin Exp Immunol. 1987;68(1):215–22.

    PubMed  CAS  Google Scholar 

  3. Bertolaccini ML, Gomez S, Pareja JF, Theodoridou A, Sanna G, Hughes GR, et al. Antiphospholipid antibody tests: spreading the net. Ann Rheum Dis. 2005;64(11):1639–43.

    Article  PubMed  CAS  Google Scholar 

  4. Mackworth-Young CG. Antiphospholipid syndrome: multiple mechanisms. Clin Exp Immunol. 2004;136(3):393–401.

    Article  PubMed  CAS  Google Scholar 

  5. Wilson WA, Cucurull E. Ethnic and geographic variation in antiphospholipid syndrome. In: Khamashta M (editor) Hughes syndrome: antiphospholipid syndrome. 2nd ed. Springer; 2006.

  6. Edwards CJ, Lian TY, Badsha H, Teh CL, Arden N, Chng HH. Hospitalization of individuals with systemic lupus erythematosus: characteristics and predictors of outcome. Lupus. 2003;12(9):672–6.

    PubMed  CAS  Google Scholar 

  7. Wilson WA, Gharavi AE, Koike T, Lockshin MD, Branch DW, Piette JC, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum. 1999;42(7):1309–11.

    Article  PubMed  CAS  Google Scholar 

  8. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295–306.

    Article  PubMed  CAS  Google Scholar 

  9. Montalban J, Rio J, Khamastha M, Davalos A, Codina M, Swana GT, et al. Value of immunologic testing in stroke patients. A prospective multicenter study. Stroke. 1994;25(12):2412–5.

    PubMed  CAS  Google Scholar 

  10. Nencini P, Baruffi MC, Abbate R, Massai G, Amaducci L, Inzitari D. Lupus anticoagulant and anticardiolipin antibodies in young adults with cerebral ischemia. Stroke. 1992;23(2):189–93.

    PubMed  CAS  Google Scholar 

  11. Levine SR, Brey RL, Tilley BC, Thompson JL, Sacco RL, Sciacca RR, et al. Antiphospholipid antibodies and subsequent thrombo-occlusive events in patients with ischemic stroke. JAMA. 2004;291(5):576–84.

    Article  PubMed  CAS  Google Scholar 

  12. Hughes GR. Migraine, memory loss, and “multiple sclerosis”. Neurological features of the antiphospholipid (Hughes’s) syndrome. Postgrad Med J. 2003;79(928):81–3.

    Article  PubMed  CAS  Google Scholar 

  13. Mackworth-Young CG, Hughes GR. Epilepsy: an early symptom of systemic lupus erythematosus. J Neurol Neurosurg Psychiatry. 1985;48(2):185.

    Article  PubMed  CAS  Google Scholar 

  14. Cimaz R, Meroni PL, Shoenfeld Y. Epilepsy as part of systemic lupus erythematosus and systemic antiphospholipid syndrome (Hughes syndrome). Lupus. 2006;15(4):191–7.

    Article  PubMed  CAS  Google Scholar 

  15. Lampropoulos CE, Koutroumanidis M, Reynolds PP, Manidakis I, Hughes GR, D’Cruz DP. Electroencephalography in the assessment of neuropsychiatric manifestations in antiphospholipid syndrome and systemic lupus erythematosus. Arthritis Rheum. 2005;52(3):841–6.

    Article  PubMed  Google Scholar 

  16. Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998;77(3):195–207.

    Article  CAS  Google Scholar 

  17. Font J, Cervera R. Cardiac manifestations of the antiphospholipid syndrome. In: Khamashta M (editors) Hughes syndrome: antiphospholipid syndrome. 1st ed. London: Springer; 2000.

    Google Scholar 

  18. Williams EL, Endean AL, Edwards CJ. Myocardial infarction in a young man with antiphospholipid syndrome and cocaine use. Lupus. 2007;16(6):444–6.

    Article  PubMed  CAS  Google Scholar 

  19. Nair S, Khamashta MA, Hughes GR. Syndrome X and Hughes syndrome. Lupus. 2002;11(5):332.

    Article  PubMed  CAS  Google Scholar 

  20. Christodoulou C, Zain M, Bertolaccini ML, Sangle S, Khamashta MA, Hughes GR, et al. Prevalence of an abnormal ankle-brachial index in patients with antiphospholipid syndrome with pregnancy loss but without thrombosis: a controlled study. Ann Rheum Dis. 2006;65(5):683–4.

    Article  PubMed  CAS  Google Scholar 

  21. Doria A, Shoenfeld Y, Wu R, Gambari PF, Puato M, Ghirardello A, et al. Risk factors for subclinical atherosclerosis in a prospective cohort of patients with systemic lupus erythematosus. Ann Rheum Dis. 2003;62(11):1071–7.

    Article  PubMed  CAS  Google Scholar 

  22. Sangle S, D’Cruz D, Khamashta M, Tungekar MF, Abbs I, Hughes G. Goldblatt’s kidney, Hughes syndrome and hypertension. Lupus. 2002;11(11):699–703.

    Article  PubMed  CAS  Google Scholar 

  23. Asherson RA, Hughes GR. Addison’s disease and primary antiphospholipid syndrome. Lancet. 1989;2(8667):874.

    Article  PubMed  CAS  Google Scholar 

  24. Sangle S, Jan W, Lau I, Bennett A, Rankin SC, Hughes GR. Coeliac artery stenosis in patients with antiphospholipid syndrome/antiphospholipid antibodies. Arthritis Rheum. 2005;52:S569.

    Google Scholar 

  25. Mangia A, Margaglione M, Cascavilla I, Gentile R, Cappucci G, Facciorusso D, et al. Anticardiolipin antibodies in patients with liver disease. Am J Gastroenterol. 1999;94(10):2983–7.

    Article  PubMed  CAS  Google Scholar 

  26. Toubi E. The ear and antiphospholipid syndrome. In: Khamashta M (editors) Hughes syndrome: antiphospholipid syndrome. 1st ed. London: Springer; 2000.

    Google Scholar 

  27. Asherson RA, Mackworth-Young CG, Boey ML, Hull RG, Saunders A, Gharavi AE, et al. Pulmonary hypertension in systemic lupus erythematosus. Br Med J (Clin Res Ed). 1983;287(6398):1024–5.

    CAS  Google Scholar 

  28. Bizzaro N, Brandalise M. EDTA-dependent pseudothrombocytopenia. Association with antiplatelet and antiphospholipid antibodies. Am J Clin Pathol. 1995;103(1):103–7.

    PubMed  CAS  Google Scholar 

  29. Macchi L, Rispal P, Clofent-Sanchez G, Pellegrin JL, Nurden P, Leng B, et al. Anti-platelet antibodies in patients with systemic lupus erythematosus and the antiphospholipid syndrome: their relationship with the observed thrombocytopneia. Br J Haematol. 1997;98(2):336–41.

    Article  PubMed  CAS  Google Scholar 

  30. Montecucco C, Caporali R. Haemocytopaenias in antiphospholipid syndrome. In: Khamashta M (editor). Hughes syndrome: antiphospholipid syndrome. 2nd ed. London: Springer; 2007.

    Google Scholar 

  31. Moore J, Ma DD, Concannon A. Non-malignant bone marrow necrosis: a report of two cases. Pathology. 1998;30(3):318–20.

    Article  PubMed  CAS  Google Scholar 

  32. Vasoo S, Sangle S, Zain M, D’Cruz D, Hughes G. Orthopaedic manifestations of the antiphospholipid (Hughes) syndrome. Lupus. 2005;14(5):339–45.

    Article  PubMed  CAS  Google Scholar 

  33. Sangle S, D’Cruz DP, Khamashta MA, Hughes GR. Antiphospholipid antibodies, systemic lupus erythematosus, and non-traumatic metatarsal fractures. Ann Rheum Dis. 2004;63(10):1241–3.

    Article  PubMed  CAS  Google Scholar 

  34. Williams FM, Chinn S, Hughes GR, Leach RM. Critical illness in systemic lupus erythematosus and the antiphospholipid syndrome. Ann Rheum Dis. 2002;61(5):414–21.

    Article  PubMed  CAS  Google Scholar 

  35. Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530–4.

    Article  PubMed  CAS  Google Scholar 

  36. Derksen RH, Khamashta MA, Branch DW. Management of the obstetric antiphospholipid syndrome. Arthritis Rheum. 2004;50(4):1028–39.

    Article  PubMed  CAS  Google Scholar 

  37. Letellier E, Hughes GR. “Listen to the patient”–anticoagulation is critical in the antiphospholipid (Hughes’s) syndrome. J Rheumatol. 2003;30(4):897.

    PubMed  Google Scholar 

  38. Veneri D, Ambrosetti A, Franchini M, Mosna F, Poli G, Pizzolo G. Remission of severe antiphospholipid syndrome associated with non-Hodgkin’s B-cell lymphoma after combined treatment with rituximab and chemotherapy. Haematologica. 2005;90(Suppl):ECR37.

    PubMed  Google Scholar 

  39. Rubenstein E, Arkfeld DG, Metyas S, Shinada S, Ehresmann S, Liebman HA. Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol. 2006;33(2):355–7.

    PubMed  Google Scholar 

  40. Sherer Y, Shoenfeld Y. Intravenous immunoglobulin for immunomodulation of systemic lupus erythematosus. Autoimmun Rev. 2006;5(2):153–5.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Rheumatology, Southampton University Hospitals, Southampton, UK

    C. J. Edwards

  2. London Lupus Centre, London Bridge Hospital, 27-29 Tooley Street, London, SE1 2PR, UK

    G. R. V. Hughes

Authors
  1. C. J. Edwards
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  2. G. R. V. Hughes
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Correspondence to G. R. V. Hughes.

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Edwards, C.J., Hughes, G.R.V. Hughes syndrome (the antiphospholipid syndrome): 25 years old. Mod Rheumatol 18, 119–124 (2008). https://doi.org/10.1007/s10165-008-0042-3

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  • DOI: https://doi.org/10.1007/s10165-008-0042-3

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