Síndrome antifosfolípido
- Bohórquez Heras, C. 1
- Pérez Gómez, A. 1
- Movasat Hajkhan, A. 1
- Rabadán Rubio, E. 1
- 1 Servicio de Enfermedades del Sistema Inmune-Reumatología, Hospital Príncipe de Asturias, Alcalá de Henares, Madrid, España
ISSN: 0304-5412
Año de publicación: 2021
Título del ejemplar: Enfermeades del sistema inmune (IV)
Serie: 13
Número: 31
Páginas: 1760-1768
Tipo: Artículo
Otras publicaciones en: Medicine: Programa de Formación Médica Continuada Acreditado
Resumen
Antiphospholipid syndrome (APS) is a condition characterised by the presence of venous and/or arterial thrombosis, together with obstetric disease, and is the major cause of acquired thrombophilia. Antiphospholipid antibodies are implicated in its pathogenesis, causing a prothrombotic state in the patient. Classification criteria have been developed for APS and for catastrophic APS, a rare but very severe form of the disease with a very poor prognosis. The clinical picture can be very varied, depending on the vascular territory affected. In addition, manifestations not included in the classification criteria such as skin lesions or thrombopenia may be found in some patients. Anticoagulation is the mainstay of treatment, although in some cases it may be necessary to use steroids or biological drugs such as rituximab, as in the case of catastrophic APS. Primary prophylaxis will depend on the patient's clinical situation.
Referencias bibliográficas
- Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306.
- Cervera R. Antiphospholipid syndrome. Thromb Res. 2017;151 Suppl1:S43-S47.
- Palomo I, Segovia F, Ortega C, Pierangeli S. Antiphospholipid syndrome: a comprehensive review of a complex and multisystemic disease. Clin Exp Rheumatol. 2009;27:668-77.
- Chighizola CB, Raschi E, Banzato A, Borghi MO, Pengo V, Meroni PL. The challenges of lupus anticoagulants. Expert Rev Hematol. 2016;9(4):389-400.
- Giannakopoulos B, Krilis SA. The pathogenesis of the antiphospholipid syndrome. N Engl J Med. 2013;368:1033-44.
- Ruiz Irastorza G, Crowther M, Branch W, Khamashta MA. Antiphospholipid syndrome. Lancet. 2010;376(9751):1498-509.
- López Pedrera C, Buendía P, Cuadrado MJ, Siendones E, Aguirre MA, Barbarroja N. Antiphospholipid antibodies from patients with the antiphospholipid syndrome induce monocyte tissue factor expression through the simultaneous activation of NF-kappaB/Rel proteins via the p38 mitogen activated protein kinase pathway, and of the MEK-1/ERK pathway. Arthritis Rheum. 2006;54:301-1.
- Cervera R, Font J, Gómez Puerta JA, Espinosa G, Cucho M, Bucciarelli S. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis. 2005;64:1205-9.
- Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT. Euro Phospholipid Project Group. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46:1019-27.
- Sciascia S, López Pedrera C, Roccatello D, Cuadrado M. Catastrophic antiphospholipid syndrome (CAPS). Best Pract Res Clin Rheumatol. 2012 Aug;26(4):535-41.
- Cervera R, Bucciarelli S, Plasín MA, Gómez Puerta JA, Plaza J, Pons Estel G. Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum On Antiphospholipid Antibodies). Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the «CAPS Registry». J Autoinmun. 2009;32:240-5.
- Rodríguez Pinto I, Espinosa G, Cervera R. Catastrophic APS in the context of other thrombotic microangiopathies. Curr Rheumatol Rep. 2015;17:482.
- Cuadrado Lozano MJ, Marenco de la Fuente JL. Lupus eritematoso sistémico (II). Pronóstico. Tratamiento. Síndrome antifosfolipídico. PDR. SER 2019.
- Sobrino Grande C, Villalobos Sánchez L, Valero Expósito M, García Villanueva MJ. Síndrome antifosfolípido. Medicine. 2017;12(27):1551-9.
- Cáliz Cáliz R, Díaz del Campo Fontecha P, Galindo Izquierdo M, López Longo FJ, Martínez Zamora MÁ, Santamaría Ortiz A. Recommendations of the Spanish Rheumatology Society for Primary Antiphospholipid Syndrome. Part I: Diagnosis, evaluation and treatment. Reumatol Clin. 2020;16(2 Pt 1):71-86.
- Pengo V, Ruffatti A, Legnani C, Gresele P, Barcellona D, Erba N. Clinical course of high risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost. 2010;8:237-42.
- Sciascia S, Khamashta MA, Bertolaccini ML. New tests to detect antiphospholipid antibodies: Antiprothrombin (aPT) and antiphosphatidyl serine/prothrombin (aPS/PT) antibodies. Curr Rheumatol Rep. 2014;16:415.
- Bertolaccini ML, Amengual O, Andreoli L, Atsumi T, Chighizola CB, Forastiero R. 14th International Congress on Antiphospholipid Antibodies Task Force Report on antiphospholipid syndrome laboratory diagnostics and trends. Autoimmun Rev. 2014;13:917-30.
- Cuadrado MJ, Bertolaccini ML, Seed PT, Tektonidou MG, Aguirre A, Mico L. Low dose aspirin vs low dose aspirin plus low intensity warfarin in thromboprophylaxis: a prospective, multicentre, randomized, open, controlled trial in patients positive for antiphospholipid antibodies (ALIWAPAS). Rheumatology (Oxford). 2014;53(2):275-84.
- Pengo V, Ruffatti A, Legnani C, Testa S, Fierro T, Marongiu F. Incidence of a first thromboembolic event in asymptomatic carriers of high risk antiphospholipid antibody profile: a multicenter prospective study. Blood. 2011;118:4714-8.
- Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat Chalumeau N. EULAR recommendations for the management of antiphospholipid síndrome in adults. Ann Rheum Dis. 2019;78:1296-304.
- Radin M, Schreiber K, Costanzo P, Cecchi I, Roccatello D, Baldovino S. The adjusted Global AntiphosPholipid Syndrome Score (aGAPSS) for risk stratification in young APS patients with acute myocardial infarction. Int J Cardiol. 2017;240:72-7.
- Radin M, Sciascia S, Erkan D, Pengo V, Tektonidou MG, Ugarte A. APS ACTION. The adjusted global antiphospholipid syndrome score (aGAPSS) and the risk of recurrent thrombosis: Results from the APS ACTION cohort. Semin Arthritis Rheum. 2019;49(3):464-8.
- Schmidt Tanguy A, Voswinkel J, Henrion D, Subra JF, Loufrani L, Rohmer V. Antithrombotic effects of hydroxychloroquine in primary antiphospholipid syndrome patients. J Thromb Haemost. 2013;11:1927-9.
- Erkan D, Willis R, Murthy VL, Basra G, Vega J, Ruiz Limon P. A prospective open label pilot study of fluvastatin on proinflammatory and prothrombotic biomarkers in antiphospholipid antibody positive patients. Ann Rheum Dis. 2014;73:1176-80.
- Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffatti A. Rivaroxaban vs warfarin in high risk patients with antiphospholipid syndrome. Blood. 2018;32:1365-71.
- Cáliz Cáliz R, Díaz Del Campo Fontecha P, Galindo Izquierdo M, López Longo FJ, Martínez Zamora MÁ, Santamaria Ortiz A. Recomendaciones de la sociedad española de Reumatología sobre el síndrome antifosfolípido primario. Parte II. Síndrome antifosfolípido obstétrico y situaciones especiales. Reumatol Clin. 2020;16(2):133-48.
- Latino JO, Perés Wingeyer SDA, Fernández Romero DS, de Larrañaga GF. Pregnancy failure in patients with obstetric antiphospholipid síndrome with conventional treatment: The influence of a triple positive antibody profile. Lupus. 2017;26:983-8.
- Tenti S, Cheleschi S, Guidelli GM, Galeazzi M, Fioravanti A. Intravenous immunoglobulins and antiphospholipid syndrome: How, when and why? A review of the literature. Autoimmun Rev. 2016 Mar;15(3):226-35.
- Ruffatti A, Favaro M, Hoxha A, Zambon A, Marson P, del Ross T. Apheresis and intravenous immunoglobulins used in addition to conventional therapy to treat high risk pregnant antiphospholipid antibody syndrome patients. A prospective study. J Reprod Immunol. 2016;115:14-915:226-35.
- Espinosa GRPI, Erkan D, Shoenfeld Y, Cervera R, on behalf of the CAPS Registry Project Group (European Forum on Antiphospholipid Antibodies). CO38 El efecto de la triple terapia con anticoagulación, corticoides, recambios plasmáticos y/o inmunoglobulinas intravenosas sobre la mortalidad de los pacientes con síndrome antifosfolípido catastrófico. Reuma-tol Clin. 2017;13SuplC:1-28.