Autonomic dysfunction and microvascular damage in systemic sclerosis.

Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular damage and interstizial fibrosis of many organs. Our interest was focused on the evaluation of cardiac autonomic function by measurements of heart rate variability (HRV) and microvascular damage detected by nailfold capillaroscopy (NC) in SSc patients. We examined 25 consecutive outpatients affected by systemic sclerosis and 25 healthy controls. Exclusion criteria were the presence of cardiac disease, hypertension, diabetes mellitus, or neurological diseases. All subjects underwent 24-h ambulatory ECG Holter recording and NC examination. Heart rate variability was evaluated in the time domain, using appropriate software, computing the time series of all normal-to-normal (NN) QRS intervals throughout the 24-h recording period. A semiquantitative rating scale was adopted to score the NC abnormalities, as well as a rating system for avascular areas and morphological NC patterns. In SSc patients, HRV analysis showed significantly lower values of SDNN (standard deviation of all NN intervals) (p=0.009), SDANN (standard deviation of the averages of NN intervals in all 5-min segments of the entire recording) (p=0.01), and pNN50 (the percentage of adjacent NN intervals that differed by more than 50 ms) (p=0.02), compared to the control group. These parameters in SSc patients significantly decreased with the worsening of semiquantitative capillaroscopy score. In conclusion, an abnormal autonomic nervous control of the heart might contribute to identify subclinical cardiac involvement in SSc patients. The coexistence of autonomic dysfunction with a more severe microvascular damage could be considered a potential prognostic tool in the identification of those patients particularly at risk for cardiac mortality.

Association between human leukocyte antigen (HLA) and interferon- induced thyroid diseases in four patients with HCV-related chronic hepatitis.

OBJECTIVES: The interferon-alpha (IFN-alpha) therapy for HCV hepatitis may exacerbate or induce underlying thyroid disorders. Besides viral factors, the human leukocyte antigen (HLA) may be an independent risk factor. METHODS: We evaluated fifteen patients with HCV chronic hepatitis during a period of 40 months. At the enrollment, all the patients were negative for thyroid disorders, excluding one patient with subclinical hypothyroidism. Eleven patients received IFN-alpha therapy. The HLA system was examined in every patient, evaluating antigens (n=40) of locus A, B and Cw and alleles (n=19) of locus DRB1* and DQB1*. The HLA system was also examined in healthy subjects (n=107) as a control group. RESULTS: The HCV genotype distribution in patients was: 1b=20%, 2a=60%, 3a=20%. Four IFN-treated patients presented clinical thyroid disorders, including autoimmune hypothyroidism (n=2), transient thyrotoxicosis (n=1) and subacute thyroiditis (n=1). The HLA susceptibility to thyroid disorders (antigen/allele frequency) in the whole group of patients was not different in respect to controls and normal Italian population. The patients with HCV chronic hepatitis that developed thyroid diseases after IFN- treatment had a double and specific association with the HLA system (Mantel-Haenszel X(c)(2)=4.706, p<0.05). CONCLUSIONS: This case report suggests that HLA system examination is an important and promising diagnostic aspect that may be considered in order to evaluate the appearance of thyroid disorders during the IFN-alpha treatment for HCV-related chronic hepatitis.

Attitudes and approach to cardiovascular risk factors in Italy: results of an electronic questionnaire survey.

BACKGROUND: The advent of computer-based technology has led to innovative epidemiological research methods to exploit the advantages of computer-mediated communications. The aim of the present study was to develop and evaluate a self-administered electronic questionnaire for acquiring information on cardiovascular health, knowledge and behaviours in a representative, stratified sample of the Italian population. METHODS: We report information on the attitudes and approach to cardiovascular disease prevention in a representative sample of Italian families who were interviewed at home by electronic questionnaires. The panel of families is currently used for national opinion polls and marketing surveys. Electronic questionnaires were filled out by 1683 males and 1736 females during a weekend period. RESULTS: Two-thirds of respondents reported having their blood pressure measured while only half reported having blood lipid and glucose tests over the previous 2 years. Prevalence of reported hypertension, hypercholesterolaemia, diabetes and smoking were 15.2, 13.0, 9.8 and 37.1% in men and 10.1, 8.1, 2.6 and 28.0% in women, respectively. More than 50% of hypertensives and diabetics were on drug treatment, while only 20% of subjects reporting hyperlipidaemia were on medication. CONCLUSIONS: The results suggest the usefulness of self-administered electronic questionnaires for acquiring quick, low-cost and high response rate information in epidemiological surveys.

Tumor necrosis factor-alpha and myocardial function in patients with myotonic dystrophy type 1.

An imbalance of TNF system activity has been reported in patients with myotonic dystrophy type 1 (DM1). Nevertheless, the question whether TNF-alpha action is directly implicated in the pathogenesis of DM1 or is a simple marker of disease activity is still open. Therefore, the present study was aimed to investigate serum tumor necrosis factor (TNF)-alpha, interleukin (IL)-1beta, and IL-6 levels in association with the disease stage, cytosine-thymine-guanine (CTG) expansion and cardiac function of 56 patients with DM1 (40+/-14 years) and 28 healthy controls (42+/-12 years). All subjects were submitted to resting electrocardiogram (EKG), Signal-averaged EKG (SA-EKG), and M-mode/2-D echocardiography. TNF-alpha levels were higher in patients compared to controls (p<0.0003) and were associated to disease stage (p<0.02). Significant correlation were observed between TNF and CTG expansion (p<0.005) or PQ intervals (p<0.0005). Ventricular late potentials (VLPs) occurred in 54% of cases. In these patients, TNF-alpha levels were higher compared to those without VLPs (p<0.05). We may conclude that TNF-alpha levels might represent and adjunctive criterion for disease staging in patients with myotonic dystrophy type 1, and that elevated TNF levels in DM1 may lead to cardiac fibrosis affecting diastolic function, conduction, and automaticity.

Ventricular late potentials in systemic sclerosis: relationship with skin involvement.

OBJECTIVE: To detect the myocardial involvement in patients with systemic sclerosis (SSc) by signal averaged electrocardiography method (SA-ECG) in relationship to the skin changes. METHODS: We selected 24 SSc outpatients according to American Rheumatism Association criteria, without clinical and instrumental evidence of cardiac disease, and compared them with 24 control subjects. All patients and controls underwent SA-ECG to detect ventricular late potentials (LP). The extent and severity of skin involvement in SSc patients was detected by modified Rodnan (m-Rodnan) skin score. RESULTS: SSc patients had higher prevalence of LP compared to controls (46 versus 8%, p < 0.003). Median skin score value in the overall SSc population was 7 [mean, SD (range): 7.8, 3.2, (4-18)]. Patients with LP had a higher median skin score value compared to SSc patients without LP [median (range): 10 (6-18) versus 6 (4-9); Mann-Whitney U test 22.5, p < 0.005]. A subset analysis was also performed to verify the correlation of antibodies positivity (anti-centromere and Scl 70) pattern and the presence of LP. Our findings showed that higher values of skin score correlated with the presence of LP independent of antibody subsets. CONCLUSIONS: Our data suggest that diffuse abnormalities of the cardiac tissue detected by SA-ECG may be present, even in patients without cardiac symptoms. The relationship between LP and skin involvement in patients without clinical evidence of cardiac involvement may help detect of a subset of patients who may develop scleroderma heart disease.