Abstract
Evaluating risk profile in patients with antiphospholipid antibodies (aPL) is an unmet need. Two recently proposed risk prediction scoring systems, aPL score and global APS score (GAPSS), are useful risk quantification tools for both thrombosis and pregnancy morbidity; they change the concept of aPL from that of “diagnostic antibodies” to “risk factors” for clinical events. Because some manifestations of aPL carry a dire prognosis, and because permanent damage may occur, a damage index for APS (DIAPS) has been developed and validated. The consequences of permanent damage due to thrombosis in APS patients decrease the health-related quality of life (HRQoL).
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295–306.
Otomo K, Atsumi T, Amengual O, et al. Efficacy of the antiphospholipid score for the diagnosis of antiphospholipid syndrome and its predictive value for thrombotic events. Arthritis Rheum. 2012;64:504–12.
Sciascia S, Bertolaccini ML. Thrombotic risk assessment in APS: the Global APS Score (GAPSS). Lupus. 2014;23:1286–7.
Sciascia S, Sanna G, Murru V, et al. The global anti-phospholipid syndrome score in primary APS. Rheumatology (Oxford). 2015;54:134–8.
Oku K, Amengual O, Bohgaki T, Horita T, Yasuda S, Atsumi T. An independent validation of the global anti-phospholipid syndrome score in a Japanese cohort of patients with autoimmune diseases. Lupus. 2015;24:774–5.
Zuily S, de Laat B, Mohamed S, et al. Validity of the global anti-phospholipid syndrome score to predict thrombosis: a prospective multicentre cohort study. Rheumatology. 2015;54:2071–5.
Amigo MC, Goycochea-Robles MV, Espinosa-Cuervo G, et al. Development and initial validation of a damage index (DIAPS) in patients with thrombotic antiphospholipid syndrome (APS). Lupus. 2015;24:927–34.
Zuily S, Rat A-C, Regnault V, et al. Impairment of quality of life in patients with antiphospholipid syndrome. Lupus. 2015;24:1161–8.
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;100:530–6.
Pengo V, Biasiolo A, Pegoraro C, Cucchini U, Noventa F, Iliceto S. Antibody profiles for the diagnosis of antiphospholipid syndrome. Thromb Haemost. 2005;93:1147–52.
Gresele P, Migliacci R, Vedovati MC, et al. Patients with primary antiphospholipid antibody syndrome and without associated vascular risk factors present a normal endothelial function. Thromb Res. 2009;123:444–51.
Meroni PL, Borghi MO, Raschi E, Tedesco F. Pathogenesis of antiphospholipid syndrome: understanding the antibodies. Nat Rev Rheumatol. 2011;7:330–9.
Giannakopoulos B, Krilis SA. The pathogenesis of the antiphospholipid syndrome. NEJM. 2013;368:1033–44.
Oku K, Amengual O, Atsumi T. Pathophysiology of thrombosis and pregnancy morbidity in the antiphospholipid syndrome. Eur J Clin Investig. 2012;42:1126–35.
Bohgaki M, Atsumi T, Yamashita Y, et al. The p38 mitogen-activated protein kinase (MAPK) pathway mediates induction of the tissue factor gene in monocytes stimulated with human monoclonal anti-beta2Glycoprotein I antibodies. Int Immunol. 2004;16:1633–41.
Oku K, Amengual O, Zigon P, Horita T, Yasuda S, Atsumi T. Essential role of the p38 mitogen-activated protein kinase pathway in tissue factor gene expression mediated by the phosphatidylserine-dependent antiprothrombin antibody. Rheumatology (Oxford). 2013;52:1775–84.
Vega-Ostertag ME, Ferrara DE, Romay-Penabad Z, et al. Role of p38 mitogen-activated protein kinase in antiphospholipid antibody-mediated thrombosis and endothelial cell activation. J Thromb Haemost JTH. 2007;5:1828–34.
Pierangeli SS, Vega-Ostertag M, Harris EN. Intracellular signaling triggered by antiphospholipid antibodies in platelets and endothelial cells: a pathway to targeted therapies. Thromb Res. 2004;114:467–76.
Brandt JT, Triplett DA, Alving B, Scharrer I. Criteria for the diagnosis of lupus anticoagulants: an update. On behalf of the Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the ISTH. Thromb Haemost. 1995;74:1185–90.
Amengual O, Atsumi T, Khamashta MA, Koike T, Hughes GR. Specificity of ELISA for antibody to beta 2-glycoprotein I in patients with antiphospholipid syndrome. Br J Rheumatol. 1996;35:1239–43.
Atsumi T, Ieko M, Bertolaccini ML, et al. Association of autoantibodies against the phosphatidylserine-prothrombin complex with manifestations of the antiphospholipid syndrome and with the presence of lupus anticoagulant. Arthritis Rheum. 2000;43:1982–93.
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;68:215–22.
Oku K, Amengual O, Atsumi T. Antiphospholipid scoring: significance in diagnosis and prognosis. Lupus. 2014;23:1269–72.
Sciascia S, Sanna G, Murru V, Roccatello D, Khamashta MA, Bertolaccini ML. GAPSS: the global anti-phospholipid syndrome score. Rheumatology (Oxford). 2013;52:1397–403.
Bertolaccini ML, Amengual O, Atsumi T, et al. ‘Non-criteria’ aPL tests: report of a task force and preconference workshop at the 13th International Congress on Antiphospholipid Antibodies, Galveston, TX, USA, April 2010. Lupus. 2011;20:191–205.
Sciascia S, Cuadrado MJ, Sanna G, et al. Thrombotic risk assessment in systemic lupus erythematosus: validation of the global antiphospholipid syndrome score in a prospective cohort. Arthritis Care Res (Hoboken). 2014;66:1915–20.
Erkan D, Yazici Y, Sobel R, Lockshin MD. Functional outcome after 10 years. J Rheumatol. 2000;27:2817–2.
Shah NM, Khamashta MA, Atsumi T, Hughes GR. Outcome of patients with anticardiolipin antibodies: a 10 years follow-up of 52 patients. Lupus. 1998;7:3–6.
Cervera R, Khamashta MA, Shoenfeld Y, et al. Euro-phospholipid project group morbidity and mortality in the antiphospholipid syndrome during 5 year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2009;68:1428–32.
Mathew S, Cesario S, Symes L. Explaining “unexplained” perinatal loss. Experiences of women with antiphospholipid syndrome. J Perinat Neonat Nurs. 2008;22:293–301.
Grika EP, Ziakas PD, Zintzaras E, Moutsopoulos HM, Vlachoyiannopoulos PG. Morbidity, mortality, and organ damage in patients with antiphospholipid syndrome. J Rheumatol. 2012;39:516–23.
Barbhaiya M, Erkan D, Rodriguez-Alamaraz E, Ramón G, Vega J, Lockshin MD. Utility of the Systemic Lupus International Collaborating Clinics (SLICC) American College of Rheumatology (ACR) Damage Index for antiphospholipid antibody (aPL) positive patients. Arthritis Rheum. 2011;63 Suppl:S3.
Feinstein AR. Clinimetrics. London: Yale University Press; 1987.
Ugolini Lopes MR, Aguila LA, Tonon RC et al. SLICC and DIAPS. Potential indices for damage assessment in primary antiphospholipid syndrome (PAPS). Presented at APLA & LACA 2013, 14th International Congress on Antiphospholipid Antibodies. 18–21 Sept 2013, Rio de Janeiro.
Abreu MM, Danowski A, Wahl DG, et al. The relevance of non criteria clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Technical Task Force Report on Antiphospholipid Syndrome Clinical Features. Autoimmun Rev. 2015;14:401–4.
Amezcua-Guerra LM. Improving definitions for an index of cumulative organ damage in patients with the antiphospholipid syndrome (DIAPS). Letter to the Editor. Lupus. 2016;25:671–2.
DeSalvo KB, Bloser N, Reynolds K, He J, Muntner P. Mortality prediction with a single general self-rated health question. A meta-analysis. J Gen Intern Med. 2006;21:267–75.
Kahn SR, Hirsch A, Shrier I. Effect of postthrombotic syndrome on health-related quality of life after deep venous thrombosis. Arch Intern Med. 2002;162:1144–8.
Khanna S, Pal H, Pandey RM, Handa R. The relationship between disease activity and quality of life in systemic lupus erythematosus. Rheumatol Oxf Engl. 2004;43:1536–40.
Jolly M, Utset TO. Can disease specific measures for systemic lupus erythematosus predict patients health related quality of life? Lupus. 2004;13:924–6.
Gladman DD, Urowitz MB, Ong A, Gough J, MacKinnon A. Lack of correlation among the 3 outcomes describing SLE: disease activity, damage and quality of life. Clin Exp Rheumatol. 1996;14:305–8.
Ware J, Kosinski M, Keller D. SF-36 physical and mental health summary scales: a User’s manual. Boston: The Health Institute, New England Medical Center; 1994.
Leplège A, Ecosse E, Pouchot J. Data from general population. In: The MOS SF-36 questionnaire. User manual and interpretation guide scores. ESTEM; Paris, France 2001. p. 67–77.
Ware J, Snow K, Kosinski M. SF-36 health survey manual and interpretation guide. Boston: The Health Institute NEMC; 1993.
EuroQol Group. EuroQol – a new facility for the measurement of health-related quality of life. Health Policy Amst Neth. 1990;16:199–208.
Oliveira de Andrade DC, Borba EF, Bonfá E, Freire de Carvalho J, José da Rocha A, Carlos Maia A. Quantifying subclinical central nervous lesions in primary antiphospholipid syndrome: the role of magnetization transfer imaging. J Magn Reson Imaging JMRI. 2008;27:483–8.
Wang C, Mayo NE, Fortin PR. The relationship between health related quality of life and disease activity and damage in systemic lupus erythematosus. J Rheumatol. 2001;28:525–32.
Costa SP, Lage LV, da Mota LMH, de Carvalho JF. Fibromyalgia in primary antiphospholipid (Hughes) syndrome. Lupus. 2011;20:1182–6.
Georgopoulou S, Efraimidou S, MacLennan SJ, Ibrahim F, Cox T. Antiphospholipid (Hughes) syndrome: description of population and health-related quality of life (HRQoL) using the SF-36. Lupus. 2015;24:174–9.
Balitsky AK, Peeva V, Su J, et al. Thrombovascular events affect quality of life in patients with systemic lupus erythematosus. J Rheumatol. 2011;38:1017–9.
Brown N, Melville M, Gray D, et al. Quality of life four years after acute myocardial infarction: short form 36 scores compared with a normal population. Heart Br Card Soc. 1999;81:352–8.
van Korlaar IM, Vossen CY, Rosendaal FR, et al. The impact of venous thrombosis on quality of life. Thromb Res. 2004;114:11–8.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing AG
About this chapter
Cite this chapter
Amigo, MC. et al. (2017). Disease and Risk Measurement Criteria in Antiphospholipid Syndrome. In: Erkan, D., Lockshin, M. (eds) Antiphospholipid Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-319-55442-6_9
Download citation
DOI: https://doi.org/10.1007/978-3-319-55442-6_9
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-55440-2
Online ISBN: 978-3-319-55442-6
eBook Packages: MedicineMedicine (R0)