- Graham Hughes
- … show all 1 hide
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.Get Access
In 1983, a detailed clinical description of a new syndrome was published. This prothrombotic syndrome was initally called the anticardiolipin syndrome and subsequently the antiphospholipid syndrome (APS), or Hughes Syndrome. Almost uniquely, it results in arterial as well as venous thrombosis and is marked by the presence of circulating antiphospholipid antibodies. Clinical features are protean, ranging from peripheral deep vein thrombosis (DVT) to involvement of internal organs such as the liver, kidneys, and adrenals. Likewise, arterial thrombosis can result in life-threatening infarction of organs such as the heart.
The nervous system is frequently affected, with migraine, memory loss, balance disorders, stroke, and atypical multiple sclerosis being prominent. Other features include recurrent miscarriage, thrombocytopenia, and livedo reticularis.
More recent observations have included ischemic bone fractures, renal and celiac artery stenosis, and a possible tendency toward accelerated atherosclerosis.
The condition is seen in patients with lupus, but, significantly, occurs without associated lupus (“primary” APS)—indeed, increasing clinical recognition of Hughes Syndrome suggests that this condition will overtake lupus in prevalance.
Treatment at present is by anticoagulation. The mechanisms for thrombosis are being worked out; it has been suggested that in some situations (e.g., pregnancy loss), an inflammatory component as well as thrombosis may play a part.
- Hughes, G. R. V. (1983), Thrombosis, abortion, cerebral disease and lupus anticoagulant. Br Med J 287, 1088–1089.
- Hughes, G. R. V. (1998), Hughes Syndrome: The antiphospholipid syndrome. A historical view. Lupus 7, 1–4. CrossRef
- Sangle, S., Jan, W., Lau, I., Bennett, A., Rankin, S. C., and Hughes, G. R. V. (2005) Coeliac artery stenosis in patients with antiphospholipid syndrome-antiphospholipid antibodies. Arthritis Rheum 52, 569–596.
- Sangle, S., D’Cruz, D., Khamashta MA., and Hughes, G. R. V. (2004), Antiphospholipid antibodies, SLE and non-traumatic metatarsal fractures. Ann Rheum Dis 63, 1241–1243. CrossRef
- Hughes, G. R. V. (2003). Migraine, memory loss and ‘multiple sclerosis’. Neurological features of the antiphospholipid (Hughes) syndrome. Postgrad Med J 79, 81–83. CrossRef
- Mackworth-Young, C. G. and Hughes, G. R. V. (1985), Epilepsy: an early symptom of SLE. J Neurol Neurosurg Psych 48, 185.
- Mackworth-Young, C. G., David, J., Loizou, S., and Walport, M. J. (1987), Primary antiphospholipid syndrome: features in patients with raised anticardiolipin antibodies and in other disorders. Brit J Rheumatol 26, 94 (abstract).
- Harris, E. N., Gharavi, A. E., Boey, M. L., et al. (1983), Anticardiolipin antibodies: detection by radioimmunoassay and association with thrombosis in SLE. Lancet 2, 1211–1244. CrossRef
- Gharavi, A. E., Harris, E. N., Asherson, R. A., and Hughes, G. R. V. (1987), Isotype distribution and phospholipid specificity in antiphospholipid syndrome. Ann Rheum Dis 46, 1–6.
- Harris, E. N., Gharavi, A. E., Asherson, R. A., Boey, M. L., and Hughes, G. R. V. (1984) Cerebral infarction in SLE. Association with anticardiolipin antibodies. Clin Exp Rheumatol 2, 47–51.
- Harris, E. N., Gharavi, A. E., Hegde, U., and Hughes, G. R. V. (1985), Anticardiolipin antibodies in autoimmune thrombocytopenic purpura. Brit J Haematol 59, 231–234.
- Asherson, R. A., Mackworth-Young, C. G., Boey, M. L., and Hughes, G. R. V. (1983), Pulmonary hypertension in SLE,. Brit Med J 287, 1024–1025. CrossRef
- Asherson, R. A., Cervera, R., and Font, J., et al. (1998), Catastrophic antiphospholipid syndrome: Clinical and laboratory features of 50 patients. Medicine 77, 195–207. CrossRef
- Harris, E. N., Gharavi, A. E., Patel, S. P., and Hughes, G. R. V. (1987), Evaluation of the anticardiolipin antibody test: report of a standardisation workshop held on April 4th 1986. Clin Exp Immunol 58, 215–222.
- Bertolaccini, M. L., Gomez, S., and Pareja, J., et al. (2005), Antiphospholipid antibody tests: spreading the net. Ann Rheum Dis 64, 1639–1643. CrossRef
- Mackworth-Young, C. G. (2004), Antiphospholipid syndrome: multiple mechanisms. Clin Exp Immunol 136, 393–401. CrossRef
- Wilson, W. A., Gharavi, A. E., and Koike, T., et al. (1999), International Consensus Statement on preliminary classification criteria for definite antiphospholipid syndrome. Arthritis Rheum 42, 1309–1311. CrossRef
- Hughes, G. R. V. (2000), Off the beaten track—a clinician’s view, in Khamashta, M. A. (ed.) Hughes Syndrome: Antiphospholipid Syndrome, Springer, London.
- Montalban, J., Khamashta, M. A., and Davalos, A., et al. (1984), Value of immunological testing in stroke patients. Stroke 25, 2412–2415.
- Nencini, P., Baruffi, M. C., and Abbata, R. et al. (1992), Lupus anticoagulant and anticardiolipin antibodies in young adults with cerebral ischaemia. Stroke 23, 189–193.
- Levine, S. R., Brey, R. L., Tilley, B. C., et al. (2004). Antiphospholipid antibodies and subsequent thrombo-occlusive events in patients with ischaemic stroke. JAMA 291, 576–584. CrossRef
- Cimaz, R., Meroni, P. L., and Shoenfeld, Y. (2006). Epilepsy as part of systemic lupus erythematosus and systemic antiphospholipid syndrome (Hughes syndrome). Lupus 15, 191–194. CrossRef
- Cuadrado, M. J., Wakefield, K., Sanna, G., Khamashta, M. A., and Hughes, G. R. V. (2004), Multiple sclerosis in the antiphospholipid (Hughes) syndrome. A common differential diagnosis?. Clin Exp Rheum 22, 652–653.
- Lampropoulos, C. E., Koutroumanidis, M., Reynolds, P. P., Manidakis, I., Hughes, G. R. V., and D’Cruz, D. P. (2005), Electroencephalography in the assessment of neuropsychiatric manifestations in antiphospholipid syndrome and systemic lupus erythematosus. Arthritis Rheum 52, 841–846. CrossRef
- Font, J. and Cervera, R. (2000), Cardiac manifestations of the antiphospholipid syndrome, in Khamashta, M. A. (ed.) Hughes Syndrome: Antiphospholipid Syndrome, Springer, London.
- Nair, S., Khamashta, M. A., and Hughes, G. R. (2002), Syndrome X and Hughes Syndrome. Lupus 11, 332. CrossRef
- Doria, A., Shoenfeld, Y., and Wu, R., et al. (2003), Risk factors for subclinical atherosclerosis in a prospective cohort of patients with SLE. Ann Rheum Dis 62, 1071–1077. CrossRef
- Christodoulou, C., Zain, M., and Bertolaccini, M. (2006), Prevalence of an abnormal ankle-brachial index in patients with antiphospholipid syndrome with pregnancy loss but without thrombosis: a controlled study. Ann Rheum Dis 65, 683–684. CrossRef
- Sangle, S., D’Cruz, D. P., and Jan, W. (2003), Renal artery stenosis in the antiphospholipid (Hughes) syndrome and hypertension. Ann Rheum Dis 62, 999–1002. CrossRef
- Sangle, S., D’Cruz, D., Khamashta, M., Tungekar, M. F., Abbs, I., and Hughes, G. R. V. (2002), Goldblatt’s kidney, Hughes syndrome and hypertension. Lupus 11, 699–703. CrossRef
- Safian, R. D. and Textor, S. C. (2001), Renal artery stenosis. N Engl J Med 344, 431–442. CrossRef
- Asherson, R. A. and Hughes, G. R. V. (1989), Addison’s disease and primary antiphospholipid syndrome. Lancet 2, 874. CrossRef
- Mangia, A., Margaglione, M., and Cascavilla, I. (1999), Anticardiolipin antibodies in patients with liver disease. Am J Gastroenterol. 94, 2983–2987. CrossRef
- Toubi, E. (2000), The ear and antiphospholipid syndrome, in: Khamashta, M. A. (ed.) Hughes Syndrome: Antiphospholipid Syndrome, Springer, London.
- Bizzarro N. and Brandalise, M. (1995), EDTA-dependent pseudo-thrombocytopenia. Am J Clin Pathol 103, 103–107.
- Montecucco, C. and Caporali, R. (2006), Haemocytopenias in antiphospholipid sindrome, in: Khamashta, M. A. (ed) Hughes Syndrome: Antiphospholipid Syndrome 2nd edition. Springer, London.
- Moore, J., Ma, D., and Concannon, A. (1998), Nonmalignant bone marrow necrosis: a report of 2 cases. Pathology 30, 318–320. CrossRef
- Vasoo, S., Sangle, S., Zain, M., D’Cruz, D., and Hughes, G. R. V. (2005), Orthopaedic manifestations of the antiphospholipid (Hughes) syndrome. Lupus 14, 339–345. CrossRef
- Tsutsumi, A., Horita, T., Ohmuro, J., Atsumi, T., Ichikawa, K., and Tashiro, K. (1999), Reflex sympathetic dystrophy in a patient with antiphospholipid syndrome. Lupus 8, 471–473. CrossRef
- Bird, A. G., Lendrum, R., Asherson, R. A., and Hughes, G. R. V. (1987), Disseminated intravascular coagulation, antiphospholipid antibodies, and ischaemic necrosis of extremities. Ann Rheum Dis 46, 251–255. CrossRef
- Asherson, R. A., Cervera, R., and de Groot, P. G., et al. (2003), Catastrophic antiphospholipid syndrome. International consensus statement on classification criteria and treatment guidelines. Lupus 12, 530–534. CrossRef
- Derksen, R. H., Khamashta, M. A., and Branch, D. W. (2004), Management of the obstetric antiphospholipid syndrome. Arthritis Rheum 50, 1028–1039. CrossRef
- Wilson, W. A. and Cucurull, E. (2006), Ethnic and geographic variation in antiphospholipid syndrome, in: Khamashta, M. A. (ed.), Hughes Syndrome: Antiphospholipid Syndrome 2nd edition. Springer, London.
- Malaviya, A. (1996), Hughes syndrome: a common problem in Kuwait hospitals. Br J Rheum, 35, 1132–1136. CrossRef
- Hughes, G. R. V. and Khamashta, M. A. (2003). Seronegative antiphospholipid syndrome. Ann Rheum Dis 62, 1127. CrossRef
- Sangle, S., D’Cruz, D. P., and Hughes, G. R. V. (2005), livedo reticularis and pregnancy morbidity in patients negative for antiphospholipid antibodies. Ann Rheum Dis 64, 147–148. CrossRef
- Letellier, E. and Hughes, G. R. V. (2003), “Listen to the patient”—anticoagulation is critical in the antiphospholipid (Hughes) syndrome. J Rheumatol 30, 897.
- Sherer, Y. and Shoenfeld, Y. (2006), Intravenous immunoglobulin for immunomodulation of SLE. Autoimmunity Rev 5, 153–155. CrossRef
- George, J. and Shoenfeld, Y. (1997), The antiphospholipid (Hughes) syndrome: a crossroads of autoimmunity and atherosclerosis. Lupus 6, 559–560.
- Hahn, B. H. (2003), Systemic lupus erythematosus and accelerated atherosclerosis. N Engl J Med 349, 2379–2380. CrossRef
- Hughes syndrome
Clinical Reviews in Allergy & Immunology
Volume 32, Issue 1 , pp 3-11
- Cover Date
- Print ISSN
- Online ISSN
- Humana Press
- Additional Links
- Antiphospholipid syndrome
- Hughes Syndrome
- Industry Sectors
- Graham Hughes (1)
- Author Affiliations
- 1. London Lupus Centre, London Bridge Hospital, London, UK