We studied the prevalence and the effect of coagulation factor V Leiden mut
ation on the occurrence of thrombotic episodes in 120 Hungarian patients ha
ving systemic lupus erythematosus (SLE) with or without antiphospholipid an
tibody. The frequency of the factor V Leiden mutation in Hungarian SLE pati
ents was 13%, which is comparable with those found previously in a healthy
Caucasian population. The incidence of venous thrombosis among factor V Lei
den carriers has been found to be higher (odds ratio [OR] 1.7) than it is i
n patients without Leiden mutation (38% vs 29%). In addition, the frequency
of venous thrombosis in the heterozygous SLE patients (OR 8.4 [confidence
interval (CI) 0.8-83.9] P = 0.06) is dependent on the coexistence of other
risk factors, such as antiphospholipid antibody. Moreover, among heterozygo
us factor V SLE patients, the Leiden mutation could explain the tendency to
have significantly higher prevalence of fetal losses (OR 3.9 [CI 1.2-12.0]
P = 0.02) and higher prevalence of cerebrovascular lesions, cardiac valvul
ar abnormalities, and Raynaud's syndrome than that found in individuals wit
hout factor V Leiden mutation or those having antiphospholipid antibody. Sy
stemic lupus erythematosus patients with combined defects suffer more sever
ely from thrombosis than those with a single risk factor do, suggesting tha
t thrombophilia is a multifactorial disorder in SLE, also. Although, the fa
ctor V Leiden mutation does not seem to be a significant risk factor for ve
nous thrombosis in SLE, these data demonstrate that Leiden mutation can be
regarded as an additive thrombogenic factor providing higher predisposition
to several vasoocclusive disorders in SLE.