Síndrome antifosfolipídico en el siglo XXI / The antiphospholipid syndrome in the 21st century

El síndrome antifosfolipídico (SAF) presenta una gran variablidad clínica, con manifestaciones que pueden ir desde tromboembolia venosa a preeclampsia, pasando por trombosis arteriales o de pequeños vasos cerebrales o renales. Es muy importante un alto grado de sospecha clínica para su diagnóstico y tratamiento antes de que produzca un daño irreversible. La positividad repetida de anticoagulante lúpico (AL), anticuerpos anticardiolipina (aCL) a valores altos o AL, aCL y anti-ß2GPI de forma combinada apoyan el diagnóstico de SAF en el contexto clínico adecuado (AU)

El tratamiento se sigue basando en los medicamentos anticoagulantes y antiagregantes. Si bien existe cierta polémica al respecto del manejo ideal de las diferentes situaciones clínicas, la anticoagulación indefinida es aceptada de forma general. Nuestra recomendación es ampliar la intensidad de anticoagulación en pacientes con trombosis recidivantes y/o arteriales y asegurar un adecuado control de los factores de riesgo vascular. En pacientes embarazadas con anticuerpos antifosfolipídicos se debe llevar a cabo un seguimiento combinado médico-obstétrico, utilizar siempre aspirina a dosis bajas y añadir heparina en las pacientes con trombosis previas, historia de muerte fetal o fracaso de la aspirina en monoterapia (AU)

Antiphospholipid antibodies predict early damage in patients with systemic lupus erythematosus.

The aim of this study was to determine whether the different autoantibodies predict early damage in patients with systemic lupus erythematosus (SLE). The patients comprised a prospective inception cohort of 205 patients with SLE, 154 on follow-up for at least five years after diagnosis. Eight patients who died before the fifth year of disease course were included in analyses comprising survival. Organ damage was measured using the Systemic Lupus International Collaborating Clinics--American College of Rheumatology damage index (SDI). Endpoints were the development of some (SDI > or = 1) or severe (SDI > 2) damage at five years after diagnosis or the combined outcome 'SDI > or = 1 or death at five years'. Autoantibodies [anti-DNA, anti-Ro, anti-La, anti-Sm, anti-U1RNP, any anti-ENA and antiphospholipid (aPL)] were included in univariate and multivariate analysis. 'Age at diagnosis' was also included as an independent variable in multivariant analyses. Sapporo criteria were used to define aPL positivity. Eighty-four patients (54.5%) had accrued damage at five years, 17 patients (11.0%) having severe damage. Patients with aPL had damage in a higher proportion (63.2% any damage, 17.6% severe damage). Only aPL were related to damage in univariate analysis (P = 0.03). In logistic regression models, aPL were the only independent predictors of damage at five years (OR 1.94, 95% CI 1.01-3.73), severe damage at five years (OR 3.34, 95% CI 1.11-10.03) and increasing damage since diagnosis (OR 2.46, 95% CI 1.24-4.87). No autoantibody was a predictor of the outcome 'SDI > or = 1 or death at five years'. The conclusion was that aPL predict early damage in patients with SLE.

Homocysteine, antiphospholipid antibodies and risk of thrombosis in patients with systemic lupus erythematosus.

Cardiovascular disease is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Antiphospholipid syndrome (APS) is one of the most important causes of thrombosis in SLE. In addition, an association between hyperhomocysteinemia and increased cardiovascular risk has also been reported. Our aim is to analyse the association of thrombosis with plasma total homocysteine (ptHcy), antiphospholipid antibodies (aPL) and other vascular risk factors in SLE patients. Fasting plasma levels of ptHcy, vitamin B12, folate, total cholesterol and creatinine were measured in 117 SLE patients. Clinical and immunological data were obtained from our prospective computerized database. aPL-positivity was defined according to Sapporo criteria. There was no association between aPL and ptHcy. ptHcy was higher in patients with arterial (median 13.02 versus 10.16 micromol/L, P = 0.010) but not venous thrombosis. In the subgroup analysis, this association was only seen in aPL-negative patients. In logistic regression, aPL (OR 6.60, 95% CI 1.86-23.34) and ptHcy (OR 1.10, 95% CI 1.01-1.19) were independently associated with arterial thrombosis. However, when hypertension, smoking and plasma total cholesterol were added to the model, only aPL (OR 7.38, 95% CI 2.02-26.91) and hypertension (OR 7.70, 95% CI 2.33-25.39), but not ptHcy, remained independently related to arterial events. aPL was the only variable independently related to venous thrombosis (OR 7.68, 95% CI 1.60-36.86). ptHcy concentrations are higher in SLE patients with arterial thrombosis. No interaction between homocysteine and aPL was found. Raised ptHcy may be a marker of increased vascular risk in aPL-negative SLE patients. The role of homocysteine as a marker of vascular risk may depend on the presence of traditional risk factors, although a modest intrinsic effect cannot be entirely excluded.

Implantación de prótesis autoexpandibles sobre estenosis aórticas por arteritis de Takayasu asociada a enfermedad de Crohn / The implantation of autoexpandible prostheses in aortic stenosis by Takayasu arteritis associated with Crohn's disease

Presentamos el caso de una mujer diagnosticada de enfermedad de Crohn que desarrolló una estenosis severa de la aorta abdominal por enfermedad de Takayasu manifestada por claudicación de ambos miembros inferiores. La colocación de prótesis intravasculares autoexpandibles (stents) sobre las lesiones aórticas estenóticas resolvió el cuadro, tanto desde el punto de vista hemodinámico como clínico de forma inmediata. (AU)