2018, Número 4
<< Anterior Siguiente >>
An Med Asoc Med Hosp ABC 2018; 63 (4)
Tormenta trombótica: síndrome antifosfolípido catastrófico
Palacios MP, Monter VJE, Martínez REA, Camarena AG
Idioma: Español
Referencias bibliográficas: 44
Paginas: 299-305
Archivo PDF: 206.71 Kb.
RESUMEN
El síndrome antifosfolípido catastrófico pertenece a un grupo de enfermedades cuya característica se centra en un estado de hipercoagulabilidad extrema. Se manifiesta aproximadamente en 1% de los pacientes con síndrome antifosfolípido y su impacto en la mortalidad es de hasta 50% de los casos. Se presenta el caso de una paciente de 41 años con antecedentes de hipertensión arterial y síndrome antifosfolípido quien ingresa a la unidad de terapia intensiva por síndrome antifosfolípido catastrófico con afección a sistema nervioso central, pulmonar, renal (lesión renal aguda) y cardiovascular (insuficiencia cardiaca congestiva, insuficiencia mitral grave y miocarditis). Durante su internamiento se le brindó tratamiento para suprimir el proceso trombótico con anticoagulantes, corticosteroides, plasmaféresis, inmunoglobulina intravenosa e hidroxicloroquina así como terapia de soporte con ventilación mecánica, apoyo inotrópico y terapia de sustitución renal continua en modalidad hemodiafiltración veno-venosa continua. Ante la severidad de la insuficiencia mitral se colocó mitraclip y así el grado de insuficiencia se redujo; sin embargo, ésta es fallida por desprendimiento del clip, por lo que se realizó cambio valvular quirúrgico. Posteriormente mostró mejoría clínica tanto a nivel pulmonar, hemodinámico y renal. Presentó estatus epiléptico con evolución tórpida y defunción.
REFERENCIAS (EN ESTE ARTÍCULO)
Ortel TL, Kitchens CS, Erkan D, Brandão LR, Hahn S, James AH et al. Clinical causes and treatment of the thrombotic storm. Expert Rev Hematol. 2012; 5 (6): 653-659.
Al-Beladi FI. Catastrophic antiphospholipid syndrome presenting as fever of unknown origin. Saudi J Kidney Dis Transpl. 2012; 23 (1): 110-113.
Wisłowska M. Catastrophic antiphospholipid syndrome. Wiad Lek. 2015; 68 (3): 271-273.
Costedoat-Chalumeaua N, Arnauda L, Saadouna D, Chastre J, Lerouxa G, Cacou P et al. Le syndrome catastrophique des antiphospholipides. La Revue de Médecine Interne. 2012; 33 (4): 194-199.
Krause I, Lev S, Fraser A, Blank M, Lorber M, Stojanovich L et al. Close association between valvar heart disease and central nervous system manifestations in the antiphospholipid syndrome. Ann Rheum Dis. 2005; 64 (10): 1490-1493.
Bucciarelli S, Espinosa G, Cervera R, Erkan D, Gómez-Puerta JA, Ramos-Casals M et al. Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 2006; 54 (8): 2568-2576.
Asherson RA. The catastrophic antiphospholipid (Asherson’s) syndrome in 2004--a review. Autoimmun Rev. 2005; 4 (1): 48-54.
Rodríguez-Pintó I, Moitinho M, Santacreu I, Shoenfeld Y, Erkan D, Espinosa G et al. Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev. 2016; 15 (12): 1120-1124.
Asherson RA, Cervera R, Piette JC, Shoenfeld Y, Espinosa G, Petri MA et al. Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore). 2001; 80 (6): 355-377.
Cervera R, Bucciarelli S, Plasín MA, Gómez-Puerta JA, Plaza J, Pons-Estel G et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun. 2009; 32 (3-4): 240-245.
Cervera R. Update on the diagnosis, treatment, and prognosis of the catastrophic antiphospholipid syndrome. Curr Rheumatol Rep. 2010; 12 (1): 70-76.
Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003; 12 (7): 530-534.
Hoayek JG, Moussa HN, Rehman HA, Nasab SH, Blackwell SC, Sibai BM. Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed. J Matern Fetal Neonatal Med. 2016; 29 (24): 3950-3955.
Cervera R, Font J, Gómez-Puerta JA, Espinosa G, Cucho M, Bucciarelli S et al. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis. 2005; 64 (8): 1205-1209.
Boey ML, Colaco CB, Gharavi AE, Elkon KB, Loizou S, Hughes GR. Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anticoagulant. Br Med J (Clin Res Ed). 1983; 287 (6398): 1021-1023.
Giannakopoulos B, Krilis SA. The pathogenesis of the antiphospholipid syndrome. N Engl J Med. 2013; 368 (11): 1033-11044.
Shoenfeld Y, Blank M, Cervera R, Font J, Raschi E, Meroni PL. Infectious origin of the antiphospholipid syndrome. Ann Rheum Dis. 2006; 65 (1): 2-6.
Ortega-Hernandez OD, Agmon-Levin N, Blank M, Asherson RA, Shoenfeld Y. The physiopathology of the catastrophic antiphospholipid (Asherson’s) syndrome: compelling evidence. J Autoimmun. 2009; 32 (1): 1-6.
Raschi E, Testoni C, Borghi MO, Fineschi S, Meroni PL. Endothelium activation in the anti-phospholipid syndrome. Biomed Pharmacother. 2003; 57 (7): 282-286.
Willis R, Pierangeli SS. Pathophysiology of the antiphospholipid antibody syndrome. Auto Immun Highlights. 2011; 2 (2): 35-52.
Go EJL, O’Neil KM. The catastrophic antiphospholipid syndrome in children. Curr Opin Rheumatol. 2017; 29 (5): 516-522.
Farzaneh-Far A, Roman MJ, Lockshin MD, Devereux RB, Paget SA, Crow MK et al. Relationship of antiphospholipid antibodies to cardiovascular manifestations of systemic lupus erythematosus. Arthritis Rheum. 2006; 54 (12): 3918-3925.
Mandal B, Rathore S, Douglas AF. Catastrophic antiphospholipid syndrome presenting as dilated cardiomyopathy with bilateral branch retinal artery thrombosis. Int J Clin Pract. 2000; 54 (8): 550-551.
Teunisse CC, Kalsbeek AJ, de Vries ST, Huisman SJ, Boers JE, Breeman A et al. Reversible cardiac valvular disease in catastrophic antiphospholipid syndrome. Neth J Med. 2010; 68 (5): 215-220.
Erdozain JG, Ruiz-Irastorza G, Segura MI, Amigo MC, Espinosa G, Pomar JL et al. Cardiac valve replacement in patients with antiphospholipid syndrome. Arthritis Care Res (Hoboken). 2012; 64 (8): 1256-1260.
Girardi G, Redecha P, Salmon JE. Heparin prevents antiphospholipid antibody-induced fetal loss by inhibiting complement activation. Nat Med. 2004; 10 (11): 1222-1226.
Christman JW, Lancaster LH, Blackwell TS. Nuclear factor kappa B: a pivotal role in the systemic inflammatory response syndrome and new target for therapy. Intensive Care Med. 1998; 24 (11): 1131-1138.
Erkan D, Cervera R, Asherson RA. Catastrophic antiphospholipid syndrome: where do we stand? Arthritis Rheum. 2003; 48 (12): 3320-3327.
Uthman I, Shamseddine A, Taher A. The role of therapeutic plasma exchange in the catastrophic antiphospholipid syndrome. Transfus Apher Sci. 2005; 33 (1): 11-17.
Espinosa G, Bucciarelli S, Cervera R, Lozano M, Reverter JC, de la Red G et al. Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies. Ann Rheum Dis. 2004; 63 (6): 730-736.
Asherson RA, Cervera R, Merrill JT, Erkan D. Antiphospholipid antibodies and the antiphospholipid syndrome: clinical significance and treatment. Semin Thromb Hemost. 2008; 34 (3): 256-266.
Orbach H, Katz U, Sherer Y, Shoenfeld Y. Intravenous immunoglobulin: adverse effects and safe administration. Clin Rev Allergy Immunol. 2005; 29 (3): 173-184.
Edwards MH, Pierangeli S, Liu X, Barker JH, Anderson G, Harris EN. Hydroxychloroquine reverses thrombogenic properties of antiphospholipid antibodies in mice. Circulation. 1997; 96 (12): 4380-4384.
Kahn P, Ramanujam M, Bethunaickan R, Huang W, Tao H, Madaio MP et al. Prevention of murine antiphospholipid syndrome by BAFF blockade. Arthritis Rheum. 2008; 58 (9): 2824-2834.
Berman H, Rodríguez-Pintó I, Cervera R, Morel N, Costedoat-Chalumeau N, Erkan D et al. Rituximab use in the catastrophic antiphospholipid syndrome: descriptive analysis of the CAPS registry patients receiving rituximab. Autoimmun Rev. 2013; 12 (11): 1085-1090.
Erkan D, Salmon JE. The role of complement inhibition in thrombotic angiopathies and antiphospholipid syndrome. Turk J Haematol. 2016; 33 (1): 1-7.
Zikos TA, Sokolove J, Ahuja N, Berube C. Eculizumab induces sustained remission in a patient with refractory primary catastrophic antiphospholipid syndrome. J Clin Rheumatol. 2015; 21 (6): 311-313.
Pescador R1, Porta R, Ferro L. An integrated view of the activities of defibrotide. Semin Thromb Hemost. 1996; 22 Suppl 1: 71-75.
Amigo MC, García-Torres R. Morphology of vascular, renal, and heart lesions in the antiphospholipid syndrome: relationship to pathogenesis. Curr Rheumatol Rep. 2000; 2 (3): 262-270.
Kampolis C, Tektonidou M, Moyssakis I, Tzelepis GE, Moutsopoulos H, Vlachoyiannopoulos PG. Evolution of cardiac dysfunction in patients with antiphospholipid antibodies and/or antiphospholipid syndrome: a 10-year follow-up study. Semin Arthritis Rheum. 2014; 43 (4): 558-565.
Nochy D, Daugas E, Droz D, Beaufils H, Grünfeld JP, Piette JC et al. The intrarenal vascular lesions associated with primary antiphospholipid syndrome. J Am Soc Nephrol. 1999; 10 (3): 507-518.
Westney GE, Harris EN. Catastrophic antiphospholipid syndrome in the intensive care unit. Crit Care Clin. 2002; 18 (4): 805-817.
Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998; 77 (3): 195-207.
Vora SK, Asherson RA, Erkan D. Catastrophic antiphospholipid syndrome. J Intensive Care Med. 2006; 21 (3): 144-159.