Lege Artis Medicinae


BODOLAY Edit, SERES Ildikó, JAKAB Zsanett, CSÍPŐ István, SZILÁGYI Anna, SZEGEDI Gyula, PARAGH György

OCTOBER 18, 2006

Lege Artis Medicinae - 2006;16(10)

[INTRODUCTION - Mixed connective tissue disease (MCTD) is an inflammatory autoimmune disease with multiple organ involvement. Immune- inflammatory processes play a crucial role in the pathogenesis of atherosclerosis. The connection between inflammatory parameters and atherosclerosis in MCTD has not yet been studied. Lipid abnormality is an important risk factor of atherosclerosis. Among the lipids, HDL is protective, which is in part due to the antioxidant effect of paraoxonase. In this paper, the lipid profiles and paraoxonase activities of MCTD patients were studied and the factors causing abnormalities were investigated. PATIENTS AND METHODS - Thirty-seven patients with MCTD, who had not taken any lipid lowering drugs in the past 2 months, were enrolled in the study. Thirty healthy individuals served as controls. At the time of the study the mean age of the MCTD patients was 51.2 ± 9.5 years, and the mean disease duration was 11.0 ± 7.2 years. Paraoxonase activity was determined by spectrophotometry, lipid profiles were determined by a Cobas Integra 700 Analyser, the von Willebrand factor antigen (vWFAg) was measured by turbidimetry in platelet-poor plasma and the thrombomodulin and anti-endothelial cell antibody (AECA) measurements were carried out by ELISA methods. RESULTS - Paraoxonase activity in the MCTD patients was lower than in the control population (118.5 ± 64.6 U/l vs. 188.0 ± 77.6, p<0.001). The arylesterase activity was also significantly lower in the patients (p<0.001). The reduction of paraoxonase activity was in correlation with the age of the patients, the duration of the disease and with vascular (eye, cardiac, cerebral) disorders. The total cholesterol and triglicerid levels of the patients were significantly increased compared to the control group, while in the apoA1 levels a significant reduction was seen. A very strong correlation was observed between the reduction of paraoxonase activity and the increase of endothelial cell activation markers (thrombomodulin, vWFAg, AECA). There was no difference in the values of patients with or without corticosteroid treatment. CONCLUSIONS - The results suggest that in MCTD there is an increased risk for atherosclerosis. Apart from an elevated cholesterol and triglicerid level, a reduced paraoxonase level and activity may also play a role in the development of atherosclerosis,. Therefore, in patients with MCTD, due to the increased oxidative processes and the impaired elimination of free radicals, a sustained damage to the endothelial cells occurs, which is indicated by increased levels of thrombomodulin, vWFAg, and anti-endothelial antibody.]



Further articles in this publication

Lege Artis Medicinae


KOVÁCS Ferenc, GYÖKERES Tibor, TAKÁCS Imre Géza, PADÁNYI István, FEKETE Csaba, OROSZ Zsolt, PAP Ákos

[INTRODUCTION - The simultaneous presence of gastric mucosa-associated lymphoid tissue lymphoma (MALT) and a gastrointestinal stromal tumor (GIST) is an extremely rare finding that has not been published until now. CASE REPORT - The authors report on a 78- year-old man who was referred to their department with an emergency upper gastrointestinal bleeding. Urgent gastroscopy revealed a bleeding ulcer in the middle third of the stomach. On the follow-up endoscopy 6 weeks later, an umbilicated polypoid lesion was found proximal to the healed ulcer, which was subsequently removed by elective surgery. Histology and immunohistochemical staining of the specimen for c-Kit confirmed the diagnosis of gastrointestinal stromal tumour. A few weeks later the patient was readmitted because of haematemesis. Upon detailed histological examination of the biopsy specimens taken from the multiple superficial ulcers found near the previous lesion, MALT-lymphoma was diagnosed. The absence of Helicobacter pylori was confirmed by repeated histological examinations, serology and urea breath test. CONCLUSION - This is the first report on a patient with simultaneous presence of a gastrointestinal stromal tumour and gastric mucosaassociated lymphoid tissue lymphoma with H. pylori negativity. Several observations suggest that the development of malignant tumours of the stomach is also associated with H. pylori infection. In view of the reported case, the possibility of a common aetiology of these two neoplasms other than H. pylori infection is discussed.]

Lege Artis Medicinae

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Lege Artis Medicinae



[BACKGROUND - The main determinant of outcome in acute pancreatitis is the extent of inflammation and pancreatic necrosis. Early administration of n-3 polyunsaturated fatty acids (PUFAs) may prevent the development of severe complications through modulation of eicosanoid synthesis and cytokine release. PATIENTS AND METHODS - In the prospective, randomised clinical trial 14 patients with acute pancreatitis received n-3 PUFAs (3.3 g/day for 5- 7 days) as a supplement to their enteral formula in the form of fish oil, and another 14 patients receiving enteral nutrition served as a control group. Measurements of erythrocyte superoxidedysmutase activity, serum total antioxidant status, C-reactive protein and praealbumin concentrations were performed at admission and at day 3, 7 and 14. Beside routine laboratory and imaging examinations, the fatty acid and vitamin A and E concentrations of the serum lipid fractions were also determined at admission and at day 7 of the jejunal nutrition. The endpoints of the study were the duration of hospitalisation, the duration of jejunal nutrition and the frequency of complications. RESULTS - A significantly higher superoxidedysmutase activity was observed in patients receiving n-3 fatty acids at day 3 of the treatment. The n-3 to n-6 long chain PUFA ratio increased significantly in the serum lipids of the patients receiving n-3 PUFA supplementation, whereas remained unchanged in the controls. Supplementation resulted in a significant decrease in the length of hospitalisation (13.1±6.7 vs. 19.3±7.2 days, p<0.05) and jejunal feeding (10.6±6.7 vs. 17.6±10.5, p<0.05). Complications developed in 6/14 (42%) of the treated group and in 9/14 (64%) of the control patients. CONCLUSION - Enteral administration of n-3 PUFAs in acute pancreatitis may promote earlier recovery by moderating inflammation.]

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Hungarian Immunology

[Clinical and immunoserological characteristics of mixed connective tissue disease (MCTD) associated with pulmonary arterial hypertension (PAH)]

VÉGH Judit, CSÍPŐ István, UDVARDY Miklós, KAPPELMAYER János, LAKOS Gabriella, ALEKSZA Magdolna, ZEHER Margit, SZEGEDI Gyula, BODOLAY Edit

[INTRODUCTION - The authors investigated the clinical characteristics, survival, accumulated damage index and immunoserological abnormalities in patients with mixed connective tissue disease (MCTD) associated with pulmonary arterial hypertension (PAH). PATIENTS AND METHODS - Anti-U1RNP autoantibodies, anti-endothelial cell antibodies, anti-cardiolipin antibodies and serum trombomodulin as well as von Willebrand factor antigen concentrations were measured in 25 patients with MCTD associated with PAH (11 right heart catheterization and 14 Doppler echocardiography) and in 154 MCTD patients without PAH. Changes in arterial pulmonary pressure were followed up by echocardiography. RESULTS - In the 25 patients PAH followed MCTD diagnosis in the average 11.6±4.5 years of the diseases. The probability of survival was lower in MCTD patients with PAH than in the 154 non-PAH MCTD patients (five years survival rate in MCTD with PAH: 73.39%, vs. 96.43% in non PAH MCTD; p<0.01; 10 years survival rate 86.74% vs. 93.25%; p<0.01). Anti-endothelial cell antibodies were more frequently present in MCTD patients sera with PAH than in non PAH MCTD (p<0.001). Serum trombomodulin and vWFAg levels were higher in MCTDPAH patients than in non PAH MCTD patients (trombomodulin:34.2±15.3 ng/ml vs. 11.8±6.5 ng/ml; p<0.001; vWFAg: 311.1±147% vs. 172.5± 141%. Significant correlations were noticed between the quantity of anti-endothelial cell antibodies and serum trombomodulin level (r=0.466) as well as the quantity of anti-endothelial cell antibodies and vWFAg serum level (r=0.550). CONCLUSION - Survival probability was worse for MCTD patients with PAH than for non PAH MCTD patients. Our results suggest that in MCTD the presence of anti-endothelial cell antibodies and endothelial cell activation may play a role in the development of pulmonary arterial hypertension and in the maintenance of obliterative vascular processes.]

Hungarian Immunology

[Regulatory T cells in mixed connective tissue disease]

BARÁTH Sándor, ALEKSZA Magdolna, SZEGEDI Andrea, SIPKA Sándor, SZEGEDI Gyula, BODOLAY Edit

[INTRODUCTION - CD4+/CD25+high suppressor and IL-10 producing CD4+ regulatory T (IL-10 Treg) cells were investigated in the peripheral blood of 48 patients with mixed connective tissue disease (MCTD). Seventeen patients were in active and 31 patients in inactive state. PATIENTS AND METHODS - Measurement of the number of CD4+CD25+high suppressor and IL-10 Treg cells was carried out by flow cytometry. RESULTS - The absolute number and percent of CD4+CD25+high T cells decreased in MCTD patients compared to the healthy controls. The number of CD4+CD25+high Treg cells was lower in 17 active MCTD patients than in the inactive patients. The percent and absolute number of IL-10 Treg was elevated in the peripheral blood of patients with MCTD compared to the healthy controls. Corticosteroid and immunosuppressive drugs moved the number of regulatory T cells (CD4+CD25+high and IL-10 Treg cells) towards the normal value. CONCLUSIONS - Our results show that the decrease in the number of CD4+CD25+high T cells could play a key role in the immunoregulatory disturbance in MCTD. Elevation in the number of IL-10 Treg cells might be a compensatory mechanism to retain the balance of proinflammatory and anti-inflammatory cytokines.]

Hungarian Radiology

[Esophageal diverticula in mixed connective tissue disease]

SZÁNTÓ Dezső, SZŰCS Gabriella, DITRÓI Edit

[INTRODUCTION - The functional and morphological changes of oesophagus occur in two third of mixed connective tissue disease patients according to the literature. CASE REPORT - We report three cases of 27, 39 and 48 year old women suffering of lateral pharyngoesophageal, epibronchial and epiphrenic diverticula associated with connective tissue disease. Diverticula had an average diameter of 3.8 cm (maximal diameters: 7.2-8 cm). The esophageal pouches produced dysphagia, dystonia, motility disorders, food stagnation and vomiting, retrosternal burning sensation and tachyarrythmia after 5-16 month's latency period. In one patient pneumoesophagus also evolved. The high serum enzyme levels and proximal electromyogram proved the presence of polymyositis. CONCLUSION - The localization, number and the size of esophageal diverticula are determined by the interstitial myopathy.]