Conclusion
In conclusion, the following observations can be made. aPL are present in approximately 2% to 4% of the normal population and the prevalence increases with age. There is a high prevalence among patients with autoimmune connective tissue disorders, especially SLE. There is an association with both venous and arterial thrombosis as well as with pregnancy morbidity, but the strength of association varies amongst studies. This probably reflects different populations, study designs, and different assays and definitions used. In several studies the risk of thrombosis appears to be higher with LA and the data suggests a true association rather than epiphenomenon. In a given patient, both aCL and LA should be measured. A significant impact on long-term survival has been noted and aPL also contribute significantly to accumulated damage in diseases such as SLE. The clinical spectrum of APS features is enormous and continues to expand. It behoves us all as clinicians and health care professionals to consider an early diagnosis of Hughes syndrome, with its distinct clinical and serological features, to reduce the risk of morbidity and mortality in our patients.
Keywords
- Systemic Lupus Erythematosus
- Systemic Lupus Erythematosus Patient
- Lupus Anticoagulant
- Anticardiolipin Antibody
- Pregnancy Morbidity
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References
Cervera R, Khamashta MA, Font J, et al. Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1000 patients. Medicine (Baltimore) 1993;72:113–124.
Perez-Vazquez ME, Villa A, Drenkard C, Cabiedes J, Alarcon-Segovia D. Influence of disease duration, continued follow up and further antiphopholipid testing on the frequency and classi fication category of antiphospholipid syndrome in a cohort of patients with SLE. J Rheumatol 1993;20:437–442.
Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome. Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 2002;46:1019–1027.
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.
Lockshin MD, Sammaritano LR, Schwartzman S. Validation of the Sapporo criteria for the antiphospholipid syndrome. Arthritis Rheum 2000;43:440–443.
Alarcon-Segovia D, Deleze M, Oria CV, et al. Antiphospholipid antibodies and the antiphospholipid syndrome in SLE. A prospective analysis of 500 consecutive patients. Medicine 1989;68:353–365.
Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus erythematosus during a 10 year period. A comparison of early and late manifestations in a cohort of 1000 patients. Medicine (Baltimore) 2003;82:299–308.
Kovalevsky G, Gracia CR, Berlin JA, Sammel MD, Barnhart KT. Evaluation of the association between hereditary thrombophilias and recurrent pregnancy loss: a meta-analysis. Arch Intern Med 2004;164:558–563.
Drenkard C, Sanchez-Guerrero J, Alarcon-Segovia D. Fall in antiphospholipid antibody at time of thromboocclusive episodes in SLE. J Rheumatol 1989;16:614–617.
Perez-Vazquez ME, Cabiedes J, Cabral AR, Alarcon-Segovia D. Decrease in serum antiphospholipid antibodies upon development of the nephrotic syndrome in patients with SLE: relationship to urinary losses of IgG and other factors. Am J Med 1992;92:357–363.
Silveira LH, Jara LJ, Espinoza LR. Transient disappearance of serum antiphospholipid antibodies can also be due to prednisolone therapy. Clin Exp Rheumatol 1996;14:217–226.
McClain MT, Arbuckle MR, Heinlen LD, et al. The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus. Arthritis Rheum 2004;50:1226–1232.
Diri E, Curcurull E, Gharavi AE, et al. Antiphospholipid (Hughes) syndrome in African-American patients: IgA aCL and beta 2 glycoprotein 1 is the most frequent isotype. Lupus 1999;8:263–268.
Bertolaccini ML, Atsumi T, Amengual O, Katsumata K, Khamashta MA, Hughes GRV. IgA anticardiolipin antibody testing does not contribute to the diagnosis of antiphospholipid syndrome in patients with SLE. Lupus 1998;7(suppl 2):S184.
Fields R, Toubbeh H, Searles R, Bankhurst A. The prevalence of anticardiolipin antibodies in a healthy elderly population and its association with antinuclear antibodies. J Rheumatol 1989;16:623–625.
Antiphospholipid Antibodies in Stroke Study Group. Clinical, radiological, and pathological aspects of cerebrovascular disease associated with antiphospholipid antibodies. Stroke 1993;24(suppl 1):S1–S123.
Schved JF, Dupuy-Fons C, Biron C, Quere I, Janbon C. A prospective epidemiological study on the occurrence of antiphospholipid antibody: the Montpellier Antiphospholipid (MAP) Study. Haemostasis 1994;24:175–182.
Schulman S, Svenungsson E, Granqvist S, and the Duration of Anticoagulation Study Group. Anticardiolipin antibodies predict early recurrence of thromboembolism and death among patients with venous thromboembolism following anticoagulant therapy. Am J Med 1998;104:332–338.
Nencini P, Baruffi M, Abbate R, Massai G, Amaducci L, Inzitari D. Lupus anticoagulant and anticardiolipin antibodies in young adults with cerebral ischaemia. Stroke 1992;23:189–193.
Vaarala O, Puurunen M, Manttari M, et al. Anticardiolipin antibodies and risk of myocardial infarction in a prospective cohort of middle-aged men. Circulation 1995;91:23–27.
Kearon C, Gent M, Hirsh J, Weitz J, et al. A comparison of three months of anticoagulation with extended anticoagulation for a first episode of idiopathic venous thromboembolism. N Engl J Med 1999;340:901–907.
Wahl DG, Guillemin F, de Maistre E, Perret-Guillaume C, Lecompte T, Thibaut G. Meta-analysis of the risk of venous thrombosis in individuals with antiphospholipid antibodies without underlying autoimmune disease or previous thrombosis. Lupus 1998;7:15–22.
Ginsburg K, Liang M, Newcomer L, et al. Anticardiolipin antibodies and the risk for ischemic stroke and venous thrombosis. Ann Intern Med 1992;117:997–1002.
Janardhan V, Wolf PA, Kase CS, et al. Anticardiolipin antibodies and risk of ischemic stroke and transient ischemic attack. The Framingham cohort and offspring study. Stroke 2004;35:736–741.
Levine SR, Brey RL, Tilley BC, et al. Antiphospholipid antibodies and subsequent thrombo-occlusive events in patients with ischemic stroke. JAMA 2004;291:576–584.
Kittner S, Gorelick P. Antiphospholipid antibodies and stroke: an epidemiological perspective. Stroke 1992;23(suppl 1):1–19, 1–22.
Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus erythematosus during a 5 year period. A multicentre prospective study of 1000 patients. Medicine (Baltimore) 1999;78:167–175.
Wahl DG, Guillemin F, de Maistre E, Perret C, Lecompte T, Thibaut G. Risk for venous thrombosis related to antiphospholipid antibodies in systemic lupus erythematosus. A meta-analysis. Lupus 1997;6:467–473.
Shah NM, Khamashta MA, Atsumi T, Hughes GRV. Outcome of patients with anticardiolipin antibodies: a 10 year follow up of 52 patients. Lupus 1998;7:3–6.
Jouhikainen T, Stephansson E, Leirisalo-Repo M. Lupus anticoagulant as a prognostic marker in systemic lupus erythematosus. Br J Rheumatol 1993;32:568–573.
Soares M, Reis L, Papi JA, Cardoso CR. Rate, pattern and factors related to damage in Brazilian systemic lupus erythematosus patients. Lupus 2003;12:788–794.
Ruiz-Irastorza G, Egurbide MV, Ugalde J, Aguirre C. High impact of antiphospholipid syndrome on irreversible organ damage and survival of patients with systemic lupus erythematosus. Arch Intern Med 2004;164:77–82.
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.
Neville C, Rauch J, Kassis J, et al. Thromboembolic risk in patients with high titre anticardiolipin and multiple antiphospholipid antibodies. Thromb Haemost. 2003;90:108–115.
Khamashta M, Cuadrado M, Mujic F, Taub N, Hunt B, Hughes GRV. The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med 1995;332:993–997.
Giron-Gonzalez JA, Garcia del Rio E, Rodriguez C, Rodriguez-Martorell J, Serrano A. Antiphospholipid syndrome and asymptomatic carriers of antiphospholipid antibody: prospective analysis of 404 individuals. J Rheumatol 2004;31:1560–1567.
Roman MJ, Shanker BA, Davis A, et al. Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus. N Engl J Med 2003;349:2399–2406.
Medina G, Casaos D, Jara LJ, Vera-Lastra O, Fuentes M, Barile L, Salas M. Increased carotid artery intima-media thickness may be associated with stroke in primary antiphospholipid syndrome. Ann Rheum Dis 2003;62:607–610.
Baron M, D’Cruz DP, Khamashta MA, Hughes GRV. ABI in PAPS. Ann Rheum Dis 2005;64:144–---.
Sangle SR, D’Cruz DP, Jan W, Karim MY, Khamashta MA, Abbs IC, Hughes GR. Renal artery stenosis in the antiphospholipid (Hughes) syndrome and hypertension. Ann Rheum Dis 2003;62:999–1002.
Vaz JL, Dancour MA, Bottino DA, Bouskela E. Nailfold videocapillaroscopy in primary antiphospholipid syndrome (PAPS). Rheumatology (Oxford) 2004;43:1025–1027.
Rosendaal F. Thrombosis in the young: epidemiology and risk factors. A focus on venous thrombosis. Thromb Haemost 1997;78:1–6.
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.
Ames P, Tommasino C, D’Andrea G, Iannaccone L, Brancaccio V, Margaglione M. Thrombophilic genotypes in subjects with idiopathic antiphospholipid antibodies — prevalence and significance. Thromb Haemost 1998;79:46–49.
Peddi VR, Kant KS. Catastrophic secondary antiphospholipid syndrome with concomitant antithrombin III deficiency. J Am Soc Nephrol 1995;5:1882–1887.
Asherson RA, Khamashta MA, Ordi-Ros J, et al. The primary antiphospholipid syndrome: major clinical and serological features. Medicine 1989;68:366–374.
Soweid AM, Hajjar RR, Hewan-Lowe KO, Gonzalez EB. Skin necrosis indicating antiphospholipid syndrome in patient with AIDS. S Med J 1995;88:786–778.
Labarca J, Rabaggliati R, Radrigan F, et al. Antiphospholipid syndrome associated with cytomegalovirus infection: case report and review. Clin Infect Dis 1997;24:197–200.
Merrill JT, Shen C, Gugnani M, Lahita RG, Mongey AB. High prevalence of antiphospholipid antibodies in patients taking procainamide. J Rheumatol 1997;24:1083–1088.
Vianna JL, Khamashta MA, Ordi-Ros J, et al. Comparison of the primary and secondary antiphospholipid syndrome: a European multicenter study of 114 patients. Am J Med 1994;96:3–9.
Piette JC, Weschler B, Frances C, Papo T, Godeau P. Exclusion criteria for primary antiphospholipid syndrome. J Rheumatol 1993;20:1802–1804.
Karassa FB, Bijl M, Davies KA, et al. Role of the Fcgamma receptor IIA polymorphism in the antiphospholipid syndrome: an international meta-analysis. Arthritis Rheum 2003;48:1930–1938.
Seisdedos L, Munoz-Rodriguez F J, Cervera R, Font J, Ingelmo M. Primary antiphospholipid syndrome evolving into SLE. Lupus 1997;6:285–286.
Carbone J, Orera M, Rodriguez-Mahou M, et al. Immunological abnormalities in primary APS evolving into SLE: 6 years follow-up in women with repeated pregnancy loss. Lupus 1999;8:274–8.
Silver RM, Drapor MJ, Scott JR et al. Clinical consequences of antiphospholipid antibodies. An historic exhort study Obstet Gynecol 1994;83:372–77.
Mujic F, Cuadrado MJ, Lloyd M et al. Primary antiohospholipid syndrome evolving into SLE. J Rheumatol 1995;22:1589–1592.
Hughes GR, Khamashta MA. Seronegative antiphospholipid syndrome. Ann Rheum Dis 2003;62:1127.
Erkan D, Cervera R, Asherson RA. Catastrophic antiphospholipid syndrome: where do we stand? Arthritis Rheum 2003;48:3320–3327.
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D’Cruz, D.P. (2006). Antiphospholipid (Hughes) Syndrome: An Overview. In: Khamashta, M.A. (eds) Hughes Syndrome. Springer, London. https://doi.org/10.1007/1-84628-009-5_2
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