ABSTRACT
Objectives:
The study aimed to reveal the clinical, laboratory and serological features of patients with antiphospholipid syndrome (APS) followed in a referral center and to demonstrate the possible differences of the patients with recurrent thrombosis.
Materials and Methods:
The data of 43 patients with APS, who applied to our center between January 2010 and January 2019, were scrutinized retrospectively. Clinical, laboratory and serological features of the patients were recorded. The patients with and without recurrent thrombosis were compared. P-value <0.05 was considered as statistically significant.
Results:
Thirty-three out of 43 patients (76.6%) were female. Fourteen (32.6%) patients were classified as primary APS and 29 (67.4%) patients as secondary APS. Acute cutaneous lupus findings (53.5%), livedo reticularis (18.6%), Raynaud’s phenomenon (18.6%), and thrombocytopenia (30.2%) were the most common clinical findings, which were not included in the classification criteria. Among the females with APS, pregnancy complications occurred in 45.5%. Arterial and/or venous thrombotic events were observed in 40 (93.0%) patients. Venous and arterial thrombosis were found in 76.7% and 32.6% of the patients, respectively. Recurrent thrombosis was detected in 20 (50.0%) of the patients with thrombosis, nine of whom (20.9%) were under anticoagulant treatment. In patients with recurrent thrombosis, the disease duration was longer (p=0.004), arterial thrombosis (p=0.023) and low serum C4 levels (p=0.025) were more frequent.
Conclusion:
In this cohort of the patients with APS, we showed that the disease duration was longer, the history of arterial thrombosis and low serum C4 levels were more frequent in the patients with recurrent thrombosis than in the patients without recurrent thrombosis.
Keywords:
Antiphospholipid Syndrome, Thrombosis, Antiphospholipid Antibody, Pregnancy Morbidity
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.
2Belizna C, Stojanovich L, Cohen-Tervaert JW, et al. Primary antiphospholipid syndrome and antiphospholipid syndrome associated to systemic lupus: Are they different entities? Autoimmun Rev. 2018;17:739-745.
3Swaak AJ, van de Brink H, Smeenk RJ, et al. Incomplete lupus erythematosus: results of a multicentre study under the supervision of the EULAR Standing Committee on International Clinical Studies Including Therapeutic Trials (ESCISIT). Rheumatology (Oxford). 2001;40:89-94.
4Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12:530-534.
5Asherson RA, Cervera R. ‘Primary’, ‘secondary’ and other variants of the antiphospholipid syndrome. Lupus. 1994;3:293-298.
6Grika EP, Ziakas PD, Zintzaras E, et al. Morbidity, mortality, and organ damage in patients with antiphospholipid syndrome. J Rheumatol. 2012;39:516-523.
7Cervera 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;46:1019-1027.
8Alijotas-Reig J, Esteve-Valverde E, Ferrer-Oliveras R, et al. The European Registry on Obstetric Antiphospholipid Syndrome (EUROAPS): A survey of 1000 consecutive cases. Autoimmun Rev. 2019;18:406-414.
9Cervera R, Khamashta MA, Shoenfeld Y, et al. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2009;68:1428-1432.
10Sanna G, D’Cruz D, Cuadrado MJ. Cerebral manifestations in the antiphospholipid (Hughes) syndrome. Rheum Dis Clin North Am. 2006;32:465-490.
11Hughes GR. Migraine, memory loss, and “multiple sclerosis”. Neurological features of the antiphospholipid (Hughes’) syndrome. Postgrad Med J. 2003;79:81-83.
12Ricarte IF, Dutra LA, Abrantes FF, et al. Neurologic manifestations of antiphospholipid syndrome. Lupus. 2018;27:1404-1414.
13Cervera R, Boffa MC, Khamashta MA, et al. The Euro-Phospholipid project: epidemiology of the antiphospholipid syndrome in Europe. Lupus. 2009;18:889-893.
14de Carvalho JF, Pasoto SG, Appenzeller S. Seizures in primary antiphospholipid syndrome: the relevance of smoking to stroke. Clin Dev Immunol. 2012;2012:981519.
15Shoenfeld Y, Lev S, Blatt I, et al. Features associated with epilepsy in the antiphospholipid syndrome. J Rheumatol. 2004;31:1344-1348.
16Rovaris M, Pedroso C, Filippi M. Neuroimaging techniques in the diagnostic work-up of patients with the antiphospholipid syndrome. Curr Rheumatol Rep. 2001;3:301-306.
17Ramos-Casals M, Campoamor MT, Chamorro A, et al. Hypocomplementemia in systemic lupus erythematosus and primary antiphospholipid syndrome: prevalence and clinical significance in 667 patients. Lupus. 2004;13:777-783.
18Carbone J, Orera M, Rodríguez-Mahou M, et al. Immunological abnormalities in primary APS evolving into SLE: 6 years follow-up in women with repeated pregnancy loss. Lupus. 1999;8:274-278.
19Núñez-Álvarez CA, Hernández-Molina G, Bermúdez-Bermejo P, et al. Prevalence and associations of anti-phosphatidylserine/prothrombin antibodies with clinical phenotypes in patients with primary antiphospholipid syndrome: aPS/PT antibodies in primary antiphospholipid syndrome. Thromb Res. 2019;174:141-147.
20Saccone G, Berghella V, Maruotti GM, et al. Antiphospholipid antibody profile based obstetric outcomes of primary antiphospholipid syndrome: the PREGNANTS study. Am J Obstet Gynecol. 2017;216:525.
21Vianna 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.
22Abu-Zeinah G, Oromendia C, DeSancho MT. Thrombotic risk factors in patients with antiphospholipid syndrome: a single center experience. J Thromb Thrombolysis. 2019;48:233-239.
23Galli M, Luciani D, Bertolini G, et al. Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood. 2003;101:1827-1832.
24Pengo V, Ruffatti A, Legnani C, et al. Clinical course of high-risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost. 2010;8:237-242.
25da Silva Saraiva S, Custódio IF, de Moraes Mazetto B, et al. Recurrent thrombosis in antiphospholipid syndrome may be associated with cardiovascular risk factors and inflammatory response. Thromb Res. 2015;136:1174-1178.
26Mary-Carmen Amigo MAK. Antiphospholipid syndrome: pathogenesis, diagnosis, and management. In: Marc C. Hochberg EMG, Alan J. Silman,, Josef S. Smolen MEW, Michael H. Weisman., editors. Rheumatology (Hochberg). 7th ed. Philadelphia, PA: Elsevier.; 2019. s. 1221-1229.
27Radin M, Sciascia S, Erkan D, et al. The adjusted global antiphospholipid syndrome score (aGAPSS) and the risk of recurrent thrombosis: Results from the APS ACTION cohort. Semin Arthritis Rheum. 2019;49:464-468.
28Oku K, Atsumi T, Bohgaki M, et al. Complement activation in patients with primary antiphospholipid syndrome. Ann Rheum Dis. 2009;68:1030-1035.
29Al-khayat Z, Waheda NE, Shaker NF. Complement C3 and C4 Levels in Recurrent Aborting Women with or without Antiphospholipid and Anticardiolipin Autoantibodies. Ibnosina J Med BS. 2014;6:143-148.
30Tabacco S, Giannini A, Garufi C, et al. Complementemia in pregnancies with antiphospholipid syndrome. Lupus. 2019;28:1503-1509.
31Crowther MA, Ginsberg JS, Julian J, et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003;349:1133-1138.
32Finazzi G, Marchioli R, Brancaccio V, et al. A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost. 2005;3:848-853.