Antiphospholipid syndrome. Clinical presentation, immunological profile, and treatment
Keywords:
antiphospholipid syndrome; initial symptom; clinical manifestations with and without criteria; immunological profile; medical treatmentAbstract
Introduction: Antiphospholipid syndrome has heterogeneous clinical manifestations, specific diagnostic tools, and treatment guidelines.
Objective: To describe clinical, laboratory, immunological profile, and treatment aspects of patients with antiphospholipid syndrome.
Methods: A descriptive, cross-sectional study was conducted at the “Arnaldo Milián Castro” Hospital from 2022 to 2024 as part of an institutional doctoral project. The study population consisted of patients diagnosed with antiphospholipid syndrome and followed up at the Autoimmune Diseases Clinic from 2000 to 2015 (208), and the sample (128) was selected according to the established criteria. Clinical variables, laboratory immunological profile, and medical treatment were studied.
Results: 93.7% of the study population had systemic lupus erythematosus. The most frequent initial symptom was obstetric morbidity due to miscarriage of less than 10 weeks (30.5%). Thrombotic syndrome was the most relevant clinical manifestation meeting diagnostic criteria (84.4%), of which venous thrombosis was present (52.3%), with iliofemoral thrombosis occurring in 64.2%. Among the non-critical clinical manifestations, livedo reticularis was present in 40.6%. In the immunological profile, the lupus anticoagulant was positive in 98.4% of cases, and prednisone was the most frequently used medical treatment (83.6%).
Conclusions: The majority of patients had systemic lupus erythematosus, with miscarriage before 10 weeks of gestation as their initial symptom. The most relevant clinical manifestation was iliofemoral deep vein thrombosis, and livedo reticularis was a non-critical symptom. The lupus anticoagulant was the primary laboratory result for diagnosis, and prednisone was the most frequently used medication in treatment.
Downloads
References
1. Barbhaiya M, Zuily S, Naden R, Hendry A, Manneville F, Amigo MC, et al. 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria. Arthritis Rheumatol [Internet]. 2023; 75(10): [aprox. 7p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/37635643/
https://doi.org/10.1002/art.42624
2. Gaspar P, Sciascia S, Tektonnidou MG. Epidemiology of antiphospholipid syndrome: macro- and microvascular manifestations. Rheumatology (Oxford) [Internet]. 2024 [citado 21/03/2023];63(SI): SI24–SI36. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10846913/
https://doi.org/10.1093/rheumatology/kead571
3. Walter IJ, Klein Haneveld MJ, Lely AT, Bloemenkamp KWM, Limper M, Kooiman J. Pregnancy outcome predictors in antiphospholipid syndrome: A systematic review and meta-analysis. Autoimmunity Reviews [Internet]. 2021 [citado 21/03/ 2023];20(10): [aprox. 7 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/34280554/
https://doi.org/10.1016/j.autrev.2021.102901
4. Tumian NR, Hunt BJ. Clinical Management of Thrombotic Antiphospholipid Syndrome. J Clin Med [Internet]. 2022 [citado 20/03/2023];29(11): [aprox. 4 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/35160188/
https://doi.org/10.3390/jcm11030735
5. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thrombosis and Haemostasis [Internet]. 2006 [citado 20/03/2023];4(2): [aprox. 9 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/16420554/.
https://doi.org/10.1111/j.1538-7836.2006.01753.x
6. Hochberg MC. Updating the American college of rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum [Internet]. 1997 [citado 20/03/2023]; 40(9): [aprox. 5p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/9324032/
https://doi.org/10.1002/art.1780400928
7. Rodriguez-Pintó I, Espinosa G, Cervera R. Precision medicine and the antiphospholipid syndrome: what is the future? Clinical Rheumatology [Internet]. 2020 [citado 21/01/2023]; 39(4): [aprox. 5p.]. Disponible en: https://link.springer.com/article/10.1007/s10067-020-04987-88.8.
https://doi.org/10.1007/s10067-020-04987-8
8. Microsoft. Microsoft Excel 2016 [Internet]. Washington: USA; 2016 [citado 21/01/2023]. Disponible en: https://office.microsoft.com/excel
9. Anturis. IBM SPSS 22 [Internet]. Washington: USA; 2023 [citado 21/01/2023]. Disponible en: https://anturis.com/ibm-spss-22/
10. Abisror N, Nguyen Y, Marozio L, Esteve-Valverde E, Udry S, Pleguezuelo DE, et al. Obstetrical outcome and treatments in seronegative primary APS: data from European retrospective study. RMD Open [Internet]. 2020 [citado 21/01/2023]; 6: e001340. Disponible en: https://rmdopen.bmj.com/content/6/2/e001340
https://doi.org/10.1136/rmdopen-2020-001340
11. Medina G, Cime Ake EA, Vera-Lastra O, Saavedra MA, Cruz-Dominguez MDP, Amigo MC, et al. Damage index for antiphospholipid syndrome during long term follow-up: Correlation between organ damage accrual and quality of life. Lupus [Internet]. 2021 [citado 21/02/2023];30(1): [aprox. 7 p.]. Disponible en: https://journals.sagepub.com/doi/full/10.1177/0961203320970651
12. Serrano R, Pons-Estel GJ, Espinosa G, Quintana RM, Reverter JC, Tassies D, et al. Long-term follow-up of antiphospholipid syndrome: real-life experience from a single center. Lupus [Internet]. 2020 [citado 20/02/2023];29(9):[aprox. 9 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/32536318/
https://doi.org/10.1177/0961203320933009
13. Calderón G, Coredro Alfaro CM. CASO 3-2020: Síndrome antifosfolípido Obstétrico: Características fiiopatológicas y manejo. Revista Clínica de la Escuela de Medicina UCR- HSJD [Internet]. 2020 [citado 20/02/2023]; 10(2): [aprox. 2p.]. Disponible en: https://dialnet.unirioja.es/servlet/articulo?codigo=9479321
14. Grika EP, Ziakas PD, Zintzaras E, Moutsopoulos HM, Vlachoyiannopoulos PG. Morbidity, Mortality, and Organ Damage in Patients with Antiphospholipid Syndrome. J Rheumatol [Internet]. 2012 [citado 20/01/2023]; 39(3): [aprox. 8 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/22247356/
https://doi.org/10.3899/jrheum.110800
15. Pengo VRA, Legnani C, Gresele P, Barcellona D, Erba N, Testa S, et al. Clinical course of high-risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost [Internet]. 2010 [citado 20/01/2023]; 8(2): [aprox. 2p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/19874470/
https://doi.org/10.1111/j.1538-7836.2009.03674.x
16. Ogata Y, Fujieda Y, Sugawara M, Sato T, Ohnishi N, Kono M, et al. Morbidity and Mortality in antiphospholipid syndrome based in cluster analysis: 10 year longitudinal cohort study. Rheumatology [Internet]. 2021 [citado 20/01/2023];60(3): [aprox. 7 p.]. Disponible en: https://u-toyama.elsevierpure.com/en/publications/morbidity-and-mortality-in-antiphospholipid-syndrome-based-on-clu/
17. Liu ML, Li G, Song X, Fan Y, Li C. Prevalence, risk factors, and prognosis of central nervous system manifestations in antiphospholipid syndrom. Scientifc Reports [Internet]. 2023 [citado 21/02/2023];13(1): [aprox. 2p.]. Disponible en: https://www.nature.com/articles/s41598-023-35955-2
https://doi.org/10.1038/s41598-023-35955-2
18. Guédon AF, Catano J, Ricard L, Laurent C, de Moreuil C, Urbanski G, et al. Non-criteria manifestations in primary antiphospholipid syndrome: a French multicenter retrospective cohort study. [Internet]. 2022 [citado 20/02/2023]; 24(1): [aprox. 8 p.]. Disponible en: https://link.springer.com/article/10.1186/s13075-022-02726-9
https://doi.org/10.1186/s13075-022-02726-9
19. Toubi IK, Fraser A, Lev S, Stojanovich L, Rovensky J, Blank M, et al. Livedo reticularis is a marker for predicting multi-system thrombosis in antiphospholipid syndrome. Clin Exp Rheumatol [Internet]. 2005 [citado 20/02/2023]; 23(4): [aprox. 2p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/16095119/
https://doi.org/10.1186/s13075-022-02726-9
20. Cervera R, Serrano R, Pons-Estel GJ, Ceberio-Hualde L, Shoenfeld Y, de Ramón E, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10- year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis [Internet]. 2015 [citado 20/02/2023];74(6): [aprox. 8 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/24464962/
https://doi.org/10.1136/annrheumdis-2013-204838
21. Taraborelli M, Reggia R, Dall'Ara F, Fredi M, Andreoli L, Gerosa M, et al. Longterm outcome in PAPS. The Journal of Rheumatology [Internet]. 2017 [citado 20/02/ 2023];44(8): [aprox. 8 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/28572466/
https://doi.org/10.3899/jrheum.161364
22. Radin M, Sciascia S, Erkan D, Pengo V, Tektonidou MG, Ugarte A, et al. The Adjusted Global AntiphosPholipid Syndrome Score (aGAPSS) and the Risk of Recurrent Thrombosis: Results from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository. Semin Arthritis Rheum [Internet]. 2019 [citado 21/02/2023]; 49(3): [aprox. 2p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/31153708/
https://doi.org/10.1016/j.semarthrit.2019.04.009
23. Pires-da-Rosa G, Espinosa G, Cervera R. Non criteria manifestations of antiphosfholipid syndrome: An overwiew. Span J Med [Internet]. 2021 [citado 20/02/2023];1(2): [aprox. 12 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/20353981/
https://doi.10.1097/CM9.0000000000000705
24. Sánchez-Cano FM S-RY. Nefropatía asociada con síndrome antifosfolipídico: más allá de microangiopatía trombótica. Med Int Méx [Internet]. 2020 [citado 20/02/ 2023];36(3): [aprox. 4 p.]. Disponible en: https://medicinainterna.org.mx/article/nefropatia-asociada-con-sindrome-antifosfolipidico-mas-alla-de-microangiopatia-trombotica/
https://doi.org/10.24245/mim. v36i3.3044
25. Gómez-Puerta JA, Martín H, Amigo MC, Aguirre MA, Camps MT, Cuadrado MJ, et al. Long-Term Follow-Up in 128 Patients With Primary Antiphospholipid Syndrome Do They Develop Lupus? Medicine [Internet]. 2005 [citado 20/02/2023];84:(4): [aprox. 3p.]. Disponible en: https://journals.lww.com/md-journal/fulltext/2005/07000/long_term_follow_up_in_128_patients_with_primary.4.aspx
https://doi.org/10.1097/01.md.0000172074.53583.ea
26. Cervera R, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Kiss E, et al. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheumatic Dis [Internet]. 2009 [citado 22/02/2022];68(9): [aprox. 5 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/18801761/
https://doi.org/10.1136/ard.2008.093179
27. Del Barrio-Longarela S, Martínez-Taboada VM, Blanco-Olavarri P, Merino A, Riancho-Zarrabeitia L, Comins-Boo A, et al. Does Adjusted Global Antiphospholipid Syndrome Score (aGAPSS) Predict the Obstetric Outcome in Antiphospholipid Antibody Carriers? A Single‑Center Study. Clin Rev Allergy Immunol [Internet]. 2022 [citado 22/02/2022]; 63(2): [aprox. 3p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/34910282/
https://doi.org/10.1007/s12016-021-08915-9
28. Bustamante JG, Goyal A, Singhal M. Antiphospholipid Syndrome. StatPearls. Treasure Island (FL): StatPearls Publishing; 2021 [citado 22/02/2022]. Disponible en: https://www.ncbi.nlm.nih.gov/books/NBK430980/
29. Leal Rato M, Bandeira M, Romão VC, Aguiar de Sousa D. Neurologic Manifestations of the Antiphospholipid Syndrome -an Update. Curr Neurol Neurosci Rep [Internet]. 2021 [citado 20/02/2023];21(8): [aprox. 5p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/34125304/
https://doi.org/10.1007/s11910-021-01124-z
30. Huang S, Ninivaggi M, Chayoua W, de Laat B. Platelets and the Antiphospholipid Syndrome. Int J Mol Sci [Internet]. 2021 [citado 20/02/2023]; 22(8): [aprox. 5p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/33919627/
https://doi.org/10.3390/ijms22084200
31. Abu-Zeinah G, Oromendia C, DeSancho MT. Thrombotic risk factors in patients with antiphospholipid syndrome: a single center experience. J Thromb Thrombolysis [Internet]. 2019 [citado 20/02/2023];48(2): [aprox. 7 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/30835035/
https://doi.org/10.1007/s11239-019-01836-7
32. Mushtaq MZ, Ahsan S, Sattar Z, Zafar Mahmood SB, Amber T, Riaz M. A retrospective Review of antiphospholipid syndrome from South Asian country Arch Rheumatol [Internet]. 2022 [citado 21/02/2023];37(1): [aprox. 2p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/35949874/
https://doi.org/10.46497/archrheumatol.2022.8979
33. Chen H-H, Lin C-H, Chao W-C. Risk of Systemic Lupus Erythematosus in Patients With Anti-phospholipid Syndrome: A Population-Based Study. Front Med (Lausanne) [Internet] 2021 [citado 25/03/2023]; 8:[aprox. 2p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/34041252/
https://doi.org/10.3389/fmed.2021.654791
34. Amigo MC, Goycochea-Robles MV, Jara LJ. Development and initial validation of a damage index (DIAPS) in patients with thrombotic antiphospholipid syndrome (APS). Lupus [Internet]. 2015 [citado 22/02/2023];24(9): [aprox. 8 p.]. Disponible en: https://journals.sagepub.com/doi/10.1177/0961203315576858
https://doi.org/10.1177/0961203315576858
35. Petri MA, Avci M, Magder LS. Evaluation of different ways to identify persistent positivity of lupus anticoagulant in systemic lupus erythematosus. Lupus Sci Med [Internet]. 2020 [citado 20/03/2023];7(1): e000406. Disponible en: https://pubmed.ncbi.nlm.nih.gov/33139453/
https://doi.org/10.1136/lupus-2020-000406
36. Knight JS, Kanthi Y. Mechanisms of immunothrombosis and vasculopathy in antiphospholipid syndrome. Seminars Immunopathol [Internet]. 2022 [citado 21/02/ 2023];44: [aprox. 3p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/35122116/
https://doi.org/10.1007/s00281-022-00916-w
37. Klein Haneveld MJ, Lemmen CH, Brunekreef TE, Bijl M, Jansen AJ, de Leeuw K, et al. Diagnosis and treatment of patients with antiphospholipid syndrome: a mixed-method evaluation of care in The Netherlands. Rheumatol Advances Pract [Internet]. 2020 [citado 20/02/2023];4(2): [aprox. 10p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/32914046/
https://doi.org/10.1093/rap/rkaa021
38. Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis [Internet]. 2019 [citado 20/02/2023];78(10):[aprox. 8 p.]. Disponible en: https://pubmed.ncbi.nlm.nih.gov/31092409/
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2026 Marta María Pérez de Alejo Rodríguez, Lissette Ruiz Jorge, Eligio Barreto Fiu, Osmany Molina Concepción, Aldo de Jesús Pérez de Alejo Rodríguez

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who have publications with this journal agree to the following terms:
- Authors will retain their copyright and assign to the journal the right of first publication of their work, which will simultaneously be subject to a Creative Commons License / Attribution-Noncommercial 4.0 International (CC BY-NC 4.0) that allows third parties to share the work as long as its author and first publication in this journal are indicated.
- Authors may adopt other non-exclusive license agreements for distribution of the published version of the work (e.g., depositing it in an institutional repository or publishing it in a monographic volume) as long as the initial publication in this journal is indicated.
- Authors are allowed and encouraged to disseminate their work through the Internet (e.g., in institutional telematic archives or on their web page) before and during the submission process, which can produce interesting exchanges and increase citations of the published work. (See The effect of open access).




february 15, 2026