ABSTRACT

Objective:

In this retrospective study, we aimed to identify factors associated with increased thrombotic risk by comparing primary (P) or systemic lupus erythematosus (SLE) associated antiphospholipid syndrome (APS) patients with vascular thrombosis (VT) or pregnancy morbidity (PM).

Methods:

Demographics, clinical and laboratory characteristics of 169 patients with APS (73 PAPS and 96 SLE+APS) were obtained from the patient database. IgG and IgM isotypes of anticardiolipin and anti-β2-glycoprotein I (aβ2GPI) antibodies were measured by ELISA and lupus anticoagulant was measured by aPTT and dRVVT tests. The patients were classified as VT only, PM only or VT+PM according to their clinics. The adjusted global APS score (aGAPSS) was calculated for each patient.

Results:

47.3% of the patients had VT only, 20.1% had PM only and 32.5% had VT+PM. Fifty-nine patients had only from arterial thrombosis, 53 venous thrombosis, 21 had both and 2 patients had microvascular thrombosis. While antiphospholipid antibody profile did not differ between VT only and PM only groups, hypertension, hyperlipidemia and smoking were more prevalent in the VT only group (50.0% vs 17.6%, p=0.001; 45.0% vs 23.5%, p=0.02; 28.8% vs 11.8%, p=0,04, respectively). The prevalence of hypertension and hyperlipidemia was also higher in patients with arterial thrombosis only compared to those with venous thrombosis only (57.6% vs 32.1%, p=0.006; 54.2% vs 32.1%, p=0.015, respectively).

Conclusion:

Our results suggest that patients with thrombotic APS have a significantly higher frequency of cardiovascular risk factors and control of these may help to lessen the risk of thrombosis, especially arterial one.

References

1Miyakis 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.

2Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677-86.

3Willis R, Pierangeli SS. Pathophysiology of the antiphospholipid antibody syndrome. Auto Immun Highlights 2011;2:35-52.

4Cervera R, Serrano R, Pons-Estel GJ, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis 2015;74:1011-8.

5Artim-Esen B, Çene E, Şahinkaya Y, et al. Cluster analysis of autoantibodies in 852 patients with systemic lupus erythematosus from a single center. J Rheumatol 2014;41:1304-10.

6Ruiz-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.

7Galli M, Luciani D, Bertolini G, Barbui T. Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood 2003;101:1827-32.

8Ruffatti A, Del Ross T, Ciprian M, et al. Risk factors for a first thrombotic event in antiphospholipid antibody carriers: a prospective multicentre follow-up study. Ann Rheum Dis 2011;70:1083-6.

9Reynaud Q, Lega JC, Mismetti P, et al. Risk of venous and arterial thrombosis according to type of antiphospholipid antibodies in adults without systemic lupus erythematosus: a systematic review and meta-analysis. Autoimmun Rev 2014;13:595-608.

10Pengo V, Biasiolo A, Pegoraro C, et al. Antibody profiles for the diagnosis of antiphospholipid syndrome. Thromb Haemost 2005;93:1147-52.

11Sciascia S, Cosseddu D, Montaruli B, et al. Risk Scale for the diagnosis of antiphospholipid syndrome. Ann Rheum Dis 2011;70:1517-8.

12Sciascia S, Sanna G, Murru V, et al. GAPSS: The Global Anti-Phospholipid Syndrome Score. Rheumatology (Oxford) 2013;52:1397-403.

13Sciascia 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.

14Sciascia S, Sanna G, Murru V, et al. The global anti-phospholipid syndrome score in primary APS. Rheumatology (Oxford) 2015;54:134-8.

15Zuily 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 (Oxford) 2015;54:2071-5.

16Oku K, Amengual O, Bohgaki T, et al. An independent validation of the Global Anti-Phospholipid Syndrome Score in a Japanese cohort of patients with autoimmune diseases. Lupus 2015;24:774-5.

17Fernandez Mosteirin N, Saez Comet L, Salvador Osuna C, et al. Independent validation of the adjusted GAPSS: Role of thrombotic risk assessment in the real-life setting. Lupus 2017;26:1328-32.

18Williams B, Mancia G, Spiering W, et al. 2018 ESC/ESH Guidelines for the management of arterial hypertension. Eur Heart J 2018;39:3021-104.

19Grundy SM, Stone NJ, Bailey AL, et al. 2018 AHA/ACC/AACVPR/AAPA/ABC/ACPM/ADA/AGS/APhA/ASPC/NLA/PCNA guideline on the management of blood cholesterol: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019;73:3168-209.

20American Diabetes Association. 2. Classification and Diagnosis of Diabetes: Standards of Medical Care in Diabetes-2020. Diabetes Care 2020;43(Suppl 1):S14-31.

21Pengo V, Tripodi A, Reber G, et al. Update of the guidelines for lupus anticoagulant detection. Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost 2009;7:1737-40.

22Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002;46:1019-27.

23Cervera R, Tektonidou MG, Espinosa G, et al. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions. Lupus 2011;20:165-73.

24Matsuura E, Lopez LR. Are oxidized LDL/beta2-glycoprotein I complexes pathogenic antigens in autoimmune-mediated atherosclerosis? Clin Dev Immunol 2004;11:103-11.

25Hasunuma Y, Matsuura E, Makita Z, et al. Involvement of beta 2-glycoprotein I and anticardiolipin antibodies in oxidatively modified low-density lipoprotein uptake by macrophages. Clin Exp Immunol 1997;107:569-73.

26Di Minno MND, Scalera A, Tufano A, et al. The association of adjusted Global AntiphosPholipid Syndrome Score (aGAPSS) with cardiovascular disease in subjects with antiphospholipid antibodies. Atherosclerosis 2018;278:60-5.

27Andrade D, Bortolotto L, Bonfá E, Borba E. Primary antiphospholipid syndrome: absence of premature atherosclerosis in patients without traditional coronary artery disease risk factors. Lupus 2016;25:472-8.

28Bettiol A, Emmi G, Finocchi M, et al. Obstetric antiphospholipid syndrome is not associated with an increased risk of subclinical atherosclerosis. Rheumatology (Oxford) 2020;keaa116 (Online ahead of print).

Cardiovascular risk in antiphospholipid syndrome: A comparison of thrombotic and obstetric disease subsets

ABSTRACT

References