Predisposing factors to nonfatal cardiovascular events in women with systemic lupus erythematosus. An observational, cross-sectional, multicenter study in Spain from the risk/systemic lupus erythematosus thematic network.

Very few studies have been published on cardiovascular morbidity in Spanish patients diagnosed with systemic lupus erythematosus (SLE). Moreover, knowledge of the predictive factors for the occurrence of nonfatal events in this group of patients is scarce.This was a multicenter, observational, cross-sectional study designed to ascertain the prevalence of nonfatal cardiovascular risk factors and cardiovascular events (CVEs) in 335 Spanish women diagnosed with SLE between 2003 and 2013.The average patient age was 36.0 years (range: 26.4-45.6); 35 patients (10.7%) experienced at least 1 CVE, which most frequently affected the brain, followed by the heart, and finally, the peripheral vasculature. Both the number of admissions because of SLE (95% confidence interval [CI] odds ratio [OR] = 1.024-1.27, P = .017) and the systemic lupus international collaborating clinics (SLICC) chronicity index score (95% CI OR = 1.479-2.400, P = .000) resulted in an increase in the OR of these patients presenting a CVE. Regarding the classic risk factors, only the interaction between hypertension (HT) and treatment with antihypertensive drugs influenced the presence of CVEs (95% CI OR = 2.165-10.377, P = .000). The presence of a family history of early cardiovascular disease was also related to CVEs (95% CI OR = 2.355-40.544, P = .002). Binary logistic regression including the above factors resulted in a model in which the 3 main variables in each group persisted, implying that they must be independent of each other. However, the weight of the interaction between the family history of early cardiovascular disease and the interaction between HT and the use of antihypertensives was higher than for the number of admissions for SLE.The SLE disease activity over time (measured using the SLICC) and the number of hospital admissions due to the disease itself, both increase the risk of women with SLE presenting a CVE. Classic cardiovascular risk factors, especially HT and its treatment, as well as a family history of early CVEs, should be considered when assessing the risk of nonfatal CVEs in women with SLE.

Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients.

We conducted the current study to characterize the clinical presentation of primary Sjögren syndrome (SS) in a large cohort of Spanish patients and to determine whether epidemiologic, clinical, and analytical features modulate disease expression. Patients were from the GEMESS Study group, which was formed in 2005 and included 12 Spanish reference centers. By March 2007, the database included 1010 consecutive patients, recruited since 1994, both incident and prevalent cases. The cohort included 937 women and 73 men (ratio, 13:1), with a mean age of 53 years at diagnosis and 59 years at inclusion in the registry. Multivariate analysis showed that male patients had a lower frequency of thyroiditis, Raynaud phenomenon, and antinuclear antibodies. Young-onset patients had a low degree of sicca involvement (xerostomia and parotid enlargement) and a high frequency of immunologic markers (anti-Ro/SS-A and low C4 levels). Patients with disease duration of more than 10 years had a higher prevalence of xerophthalmia, parotid enlargement, lung involvement, and peripheral neuropathy in comparison with incident cases. The subset of patients with anti-Ro/La antibodies had the highest prevalence of most systemic, hematologic, and immunologic alterations (higher frequency of Raynaud phenomenon, altered parotid scintigraphy, positive salivary gland biopsy, peripheral neuropathy, thrombocytopenia, and rheumatoid factor). Hypocomplementemia was associated with a higher frequency of vasculitis and lymphoma, and cryoglobulins with a higher frequency of parotid enlargement, vasculitis, and leukopenia.Epidemiologic, clinical, and analytical features have a significant impact on the clinical presentation of primary SS, influencing the results of the main diagnostic tests, the prevalence and diversity of extraglandular involvement, and the frequency of the main immunologic markers. Primary SS should be considered as a systemic autoimmune disease that can express in many guises beyond sicca involvement.

Activated protein C levels in Behçet's disease and risk of venous thrombosis.

Behçet's disease is a multi-systemic inflammatory disorder of unknown cause. Most abnormalities have been associated with endothelial injury caused by vasculitis. Thrombosis occurs in about 25% of patients, although the mechanism is unknown. The objective of this study was to evaluate the protein C activation system in Behçet's disease and its correlation with venous thromboembolism (VTE). Thirty-nine patients (12 with VTE) and 78 age- and sex-matched controls were included in the study, and levels of protein C, protein S, activated protein C (APC), protein C inhibitor (PCI), soluble thrombomodulin (TM), antithrombin (AT), alpha(1)-antitrypsin, fibrinogen, factor VIII, von Willebrand factor (VWF) and C-reactive protein (CRP) were measured. APC and TM levels were significantly lower in patients than in controls, whereas protein S, AT, alpha(1)-antitrypsin, fibrinogen, factor VIII, VWF and CRP levels were significantly higher in patients than in controls. APC, PCI and TM levels were lower in patients with VTE (0.65 +/- 0.19 ng/ml, 86% +/- 22% and 15.5 +/- 7.1 ng/ml respectively) than in those without VTE (0.78 +/- 0.17 ng/ml, 100% +/- 15% and 22.1 +/- 15.3 ng/ml) (P < 0.05). In patients, APC levels below 0.75 ng/ml (10th percentile of the control group) increased the risk of VTE about fivefold (odds ratio = 5.1; 95% confidence interval = 1.1-23.4). These results show that reduced APC levels are associated with the high incidence of VTE in Behçet's disease.