Search PubMed⌕ Search

Biomedical subjects

E Bodolay

Publications and source records attributed to E Bodolay.

At least 19 recordsLinked to original sources
PubMed2006

Clinical and immunoserological characteristics of mixed connective tissue disease associated with pulmonary arterial hypertension.

We investigated the clinical characteristics and immunoserological alterations in patients with mixed connective tissue disease (MCTD) associated with pulmonary arterial hypertension (PAH). Anti-U1RNP autoantibodies, anti-endothelial cell antibodies (AECA) and serum thrombomodulin (TM) as well as von Willebrand factor antigen (vWFAg) concentrations were measured in 25 patients with MCTD associated with PAH and in 154 MCTD patients without PAH. The results showed that the probability of survival was lower in MCTD patients with PAH than in the 154 MCTD-non-PAH patients (5-year survival rate in MCTD with PAH: 73%, versus 96% in MCTD-non-PAH; P < 0.01). AECA were more frequently present in the sera of MCTD patients with PAH than in MCTD-non-PAH (P < 0.001). Serum TM and vWFAg levels were higher in MCTD-PAH patients than in MCTD-non-PAH patients (TM: P < 0.001; vWFAg: P < 0.001). Significant correlation was noticed between the quantity of AECA and TM level (r = 0.466) as well as the quantity of AECA and vWFAg level (r = 0.550). In conclusion, our results suggest that in MCTD the presence of AECA and endothelial cell activation may play a role in the development of PAH and in the maintenance of obliterative vascular processes.

Adult↗
PubMed2005

Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD).

OBJECTIVE: Interstitial lung disease (ILD) may be a characteristic, often serious, manifestation of mixed connective tissue disease (MCTD). In this retrospective study, the frequency and clinical picture of ILD were determined in patients with MCTD using two diagnostic tests: high-resolution computed tomography (HRCT) and inhaled aerosol clearance times of (99m)Tc-labelled diethylene-triamine pentaacetate ((99m)Tc-DTPA). In addition, pulmonary function, effects of therapy and a variety of immunoserological markers were also assessed. METHODS: One hundred and forty-four consecutive patients with MCTD were selected from the clinic, irrespective of the presence or absence of ILD. All patients underwent a detailed clinical assessment, chest HRCT scanning, chest radiography, inhaled aerosol of (99m)Tc-DTPA clearance times, and all pulmonary function tests. Patients who had active ILD received corticosteroid (CS) or CS in combination with cyclophosphamide (CPH). All investigations were repeated after 6 months of immunosuppressive therapy. RESULTS: Ninety-six out of 144 MCTD patients (66.6%) had active ILD, 75 of this group (78.1%) showed ground glass opacity, 21 patients (21.8%) ground glass opacity with mild fibrosis with HRCT. Forty-five patients with active ILD received 2 mg/kg/day CS for 6-8 weeks alone and 51 patients CS in combination with CPH (2 mg/kg/day). Six months later, after therapy, 67 out of 96 MCTD patients with ILD (69.8%) showed a negative HRCT pattern, ground glass opacity with mild fibrosis developed in 15 patients (15.6%), and fibrosis was detected in 13 patients (13.5%). Only one patient showed subpleural honeycombing. (99m)Tc-DTPA was rapid in all 96 MCTD patients with active ILD (28.7 +/- 8.2 min, normal value >40 min). After therapy the (99m)Tc-DTPA was normalized, 79 out of 96 patients (82.3%). Carbon monoxide diffusion capacity (DLCO) was reduced in 33 out of 96 MCTD patients with active ILD (34.3%), while there were no significant differences in the pulmonary function tests between the active versus inactive stage of ILD or versus patients without ILD. The sera of 96 MCTD patients with active ILD contained a high level of immune complexes (ICs), and the total haemolytic complement levels (CH50/ml U) decreased. After 6 months of therapy, the IC levels decreased and CH50/ml levels normalized (MCTD patients before and after active ILD: IC optical density = 355 +/- 227 vs 206 +/- 92, P<0.001; CH50/ml, 38.0 +/- 12.6 U vs 64.3 +/- 13.0 U, P<0.001). CONCLUSIONS: HRCT is the gold standard for diagnosis of ILD. However, we used another method, (99m)Tc-DTPA, in order to compare this technique with HRCT. This latter technique has not been studied previously in MCTD. The elevated levels of IC and increased complement consumption indicated that IC-mediated alveolocapillary membrane damage and tissue injury might play a role in the pathogenesis of ILD in MCTD.

Adult↗
PubMed2004

Abnormal cell-specific expressions of certain protein kinase C isoenzymes in peripheral mononuclear cells of patients with systemic lupus erythematosus: effect of corticosteroid application.

We have studied the expressions of various protein kinase C (PKC) isoenzymes in T cells and monocytes from patients with systemic lupus erythematosus (SLE), in comparison to those of healthy controls and patients with other immunological disorders. As measured by Western blotting, the levels of PKCbeta, delta, eta, epsilon, theta and zeta (but not of PKCalpha) significantly decreased in T cells of SLE patients. In monocytes, however, we observed marked suppressions only in the expressions of PKCdelta, epsilon and zeta but not in the expressions of other PKC isoforms. In vivo corticosteroid application, as well as in vitro steroid treatment of monocytes, elevated the expressions of most isoforms close to normal values; however, the decreased levels of PKCtheta and zeta were not affected by steroid application. These alterations were characteristic to SLE because we could not detect any changes in the PKC levels in mononuclear cells of primary Sjögren's syndrome and mixed connective tissue disease patients. These results suggest that impaired PKC isoenzyme pattern may exist in the T cells and monocytes of SLE patients. Furthermore, the clinically efficient glucocorticoid application in SLE can increase the expression of some members of PKC system.

Adrenal Cortex Hormones↗
PubMed2003

Osteoporosis in mixed connective tissue disease.

The existence of osteoporosis in 58 postmenopausal women with mixed connective tissue disease (MCTD) was investigated. The mean bone mineral density assessed by dual energy X-ray absorptiometry in the lumbar spine was decreased in 25.8% of the patients, reflecting osteoporosis (T score < -2.5). In the femoral neck there was no significant difference between the BMD of MCTD patients and that of age-matched, healthy postmenopausal women. Low bone mineral density was found among patients on, as well as off, corticosteroids. The extent of bone loss was associated with disease duration, as well as corticosteroid therapy. Serum osteocalcin levels were lower in MCTD patients than in controls. Lower serum oestradiol, testosterone and dehydroepiandrosterone sulphate levels were detected in MCTD patients than in controls. Thus, MCTD may be associated with increased bone loss. Pathogenic factors may include the disease itself, corticosteroid therapy, impaired osteoblast function, and low serum sex hormone levels.

Absorptiometry, Photon↗
PubMed2000

The clinical usefulness of the fingers-to-palm ratio in different hand microcirculatory abnormalities.

A non-invasive nuclear medicine technique was developed to screen patients with painful hands so as to separate patients with a normal from those with an abnormal microcirculation of the hands in different clinical conditions. Such a technique is important, as the other methods available are either subjective or rather complicated. The study population consisted of 10 healthy individuals, 23 patients with Raynaud's syndrome and 15 patients with mixed connective tissue disease (MCTD). Sixty gamma-camera images of the hands (1 s each) were recorded after a bolus injection of 99Tcm-DTPA via a dorsal foot vein. Regions of interest were drawn on the summed images around the fingers and the palmar region. The fingers-to-palm ratio was then calculated from the total counts inside these regions of interest separately for each hand. The mean fingers-to-palm ratio was 0.94+/-0.18 (0.71-1.25) for the healthy group, 0.57+/-0.22 (0.21+/-1.11) for the MCTD group and 0.40+/-0.14 (0.18-0.77) for the Raynaud's patients. Analysis of variance showed these differences to be highly significant (P < 0.001). There were also significant differences between 6 MCTD patients in an active (mean 0.48) and nine patients in an inactive (mean 0.66) clinical state (two-sample t-test: P < 0.05). There were no significant differences between the fingers-to-palm ratios of the left and right hands of the same patients (one-sample t-test). Of the 23 primary Raynaud's patients, capillary microscopic data were pathological in only eight (34%). We conclude that our method is able to differentiate between patients with normal and those with abnormal microcirculation of the hands. Although measurement of the fingers-to-palm ratio is not a specific method, it is useful both for staging and in the follow-up of patients.

Adolescent↗
PubMed2000

Hypercoagulability in various autoimmune diseases: no association with factor V Leiden mutation.

The coagulation factor V Leiden mutation, leading to resistance to activated protein C (APC), is the most common inherited risk factor for venous thrombosis. In various systemic autoimmune diseases the hypercoagulable state was shown to be associated with the presence of antiphospholipid antibodies (aPL). Our aim was to determine the prevalence of both, Leiden mutation and aPL in autoimmune diseases and their impact on the occurrence of venous thrombosis. The dataset consists of results from 137 patients having Sjögren's syndrome (n = 50), progressive systemic sclerosis (n = 43) (PSS), undifferentiated connective tissue disease (n = 24) (UCTD) and mixed connective tissue disease (n = 20) (MCTD) with or without venous thromboembolic complications. The Leiden mutation was detected with polymerase chain reaction (PCR), aPL, such as lupus anticoagulant (LA) with screening and confirmatory procedures and others with enzyme linked immunosorbent assay (ELISA). The prevalence of mutation ranged between 8.3% and 18.0% (13.1%). The thromboembolic risk was found to be increased in the presence of aPL. Eight patients (5.84%) (4 heterozygous) experienced thromboembolic events and 3 out of 4 heterozygous showed aPL positivity, too. There were no difference between the frequencies of Leiden mutation in examined systemic autoimmune diseases and unselected populations.

Activated Protein C Resistance↗
PubMed2000

Antiprothrombin and antiannexin V antibodies imply risk of thrombosis in patients with systemic autoimmune diseases.

OBJECTIVE: To investigate the relationship between antiprothrombin (aPT) and antiannexin V (aANX) autoantibodies of IgG isotype and thrombosis in patients with systemic autoimmune diseases. To compare the clinical relevance of these antibodies to that of anticardiolipin (aCL), anti-beta2-glycoprotein I antibodies (anti-beta2-GPI), and lupus anticoagulant (LAC). METHODS: Serum IgG aPT, aANX, aCL, and anti-beta2-GPI levels were measured by solid phase enzyme immunoassay in the sera of 70 patients with systemic autoimmune diseases, 35 with antiphospholipid syndrome (APS) and 35 without APS. Medical records were analyzed, and associations of the antibodies with clinical features of APS were assessed. RESULTS: Patients with APS had higher frequency of aPT (p = 0.001) and aANX (p = 0.002) compared to patients without APS. Thrombotic events occurred more frequently in those with aPT or aANX than those without (p = 0.005, p = 0.006, respectively). The presence of aPT and aANX was found to be highly specific for APS. CONCLUSION: Measurement of aPT and aANX antibodies may be of value in confirming the diagnosis of APS, and in evaluating risk of venous and arterial thrombosis in patients with systemic autoimmune diseases.

Adult↗
PubMed1999

Isotype distribution and clinical relevance of anti-beta2-glycoprotein I (beta2-GPI) antibodies: importance of IgA isotype.

The aim of this study was to evaluate the prevalence of IgG, IgA and IgM anti-beta2-GPI antibodies in anti-phospholipid syndrome (APS), and to establish the clinical significance of IgA type antibodies compared with the other isotypes. Anti-beta2-GPI antibodies were measured in the sera of 70 patients by solid-phase enzyme immunoassay in gamma-irradiated polystyrene plates coated with human purified beta2-GPI. Thirty-three out of the 70 patients were classified as having APS: three of them had primary, and 30 had secondary APS related to systemic lupus erythematosus (SLE). The remaining 37 patients had SLE without APS. Anti-beta2-GPI antibodies of IgG, IgA and IgM isotypes were present in 84.8%, 59.3% and 51.5% of patients with APS. Both the frequency and the level of each isotype were significantly higher in patients with APS. This association was very strong for IgA (P = 0.0004 for the antibody frequency and P < 0.0001 for the antibody level), as well as for IgG type antibodies (P < 0.0001 and P < 0.0001), whereas it was weaker for IgM (P = 0.01 and P = 0.04). A strong relationship was demonstrated between increased IgA anti-beta2-GPI antibody levels and a history of venous thrombosis, thrombocytopenia, heart valve disease, livedo reticularis and epilepsy. IgG anti-beta2-GPI antibodies were associated with the presence of lupus anticoagulant (LA) in addition to the main features of APS. However, antibodies of IgM isotype were related only to thrombocytopenia and heart valve disease. We recommend the evaluation of anti-beta2-GPI antibodies of IgA isotype in addition to IgG in patients with clinical suspicion of APS.

Antiphospholipid Syndrome↗
PubMed1995

Plasma endothelin correlates with antiendothelial antibodies in patients with mixed connective tissue disease.

BACKGROUND: Elevated circulating levels of the vasoactive peptide endothelin-1 have been reported in various cardiovascular disorders. Because these conditions are frequently associated with endothelial dysfunction and damage and the vasoconstrictor effect of endothelin-1 is believed to be produced at the local vascular level, it is uncertain whether circulating endothelin-1 is a causal factor in enhanced vascular tone or instead a marker of endothelial injury. METHODS AND RESULTS: We tested whether elevated immunoreactive endothelin-1 could be detected by radioimmunoassay in plasma and whether endothelin-1 levels correlated with antiendothelial autoantibodies in patients with mixed connective tissue disease. Venous blood samples were collected from 21 patients in the morning after an overnight fast and before medication. The plasma immunoreactive endothelin-1 level was 2.7 +/- 0.5 pg/mL (range, 1.1 to 5.2 pg/ml; n = 9) and 7.3 +/- 1.5 pg/mL (range, 2.8 to 20.7 pg/mL; n = 12) in patients who had no antiendothelial antibodies and in patients with antiendothelial antibodies, respectively. These latter values were significantly (P < .001) increased compared with 10 age-matched healthy volunteers (2.0 +/- 0.3 pg/mL; range, 0.5 to 3.0 pg/mL). Plasma endothelin-1 level strongly correlated with antiendothelial antibodies (rs = .836, n = 21, P < .001), whereas there was no correlation between age, systolic and diastolic blood pressures, antinuclear antibodies, and duration of the disease and endothelin-1 values. The incidence of Raynaud's phenomenon and angina did not differ significantly in patients with low and high endothelin-1 levels. CONCLUSIONS: This study showed that mixed connective tissue disease is associated with elevated plasma immunoreactive endothelin-1 and that endothelin-1 levels significantly correlate with antiendothelial autoantibodies. These findings suggest that increases in plasma endothelin-1 concentration may be secondary to vascular injury and do not necessarily represent enhanced susceptibility to vasoconstriction.

Adult↗
PubMed1991

[Primary hemostasis in mixed connective tissue disease].

Willebrand-factor antigen level and structure analysis, ristomycin-cofactor assay, beta-thromboglobulin and thromboxane metabolite estimations were performed in 22 patients with mixed connective tissue disease. High levels of Willebrand factor antigen and activity were detected in the presence of thrombocytopenia, previous thrombotic events, pulmonary vascular lesions and in the presence of circulating antiendothelial antibodies. Increased platelet activation was documented also in antibody positive cases and in thrombocytopenia. The alterations of endothelial and platelet functions may play important role in the development of vascular complications of mixed connective tissue disease.

Adult↗
PubMed1991

Alterations of primary haemostasis in mixed connective tissue disease (MCTD).

Willebrand-factor antigen level and structure analysis, ristomycin-cofactor assay, beta-thromboglobulin and thromboxane metabolite estimations were performed in 22 patients with mixed connective tissue disease to evaluate the incidence and the possible role of haemostatic alterations in the complications occurring during the course of the disease. High levels of Willebrand-factor antigen and ristomycin-cofactor activity were detected in patients with thrombocytopenia, previous thrombotic event, pulmonary vascular lesions and usually in the presence of circulating anti-endothelial antibodies. Increased platelet activation could have been found in antibody positive cases and in patients with thrombocytopenia as well. The documented alterations of endothelial and platelet functions may play important role in the vascular complications of mixed connective tissue disease.

Adult↗
PubMed1991

[Pregnancy in mixed connective tissue disease].

The authors carried out a retrospective study of the pregnancy of 31 women suffering from mixed connective tissue disease (MCTD). They summed up the number of growing children, stillborns, and spontaneous abortion in the period before and after commencement of the disease process. Their results point to a rather marked occurrence of spontaneous abortion even in the period before the actual disease process. In the majority of patients there were observable clinical signs which, retrospectively, was an autoimmune disease process, but now known as the appearance of MCTD. In the period following the disease only a smaller percentage of patients became pregnant, not on account of their age or previous spontaneous abortions, but rather because of their symptoms. They investigated those clinical abnormalities which could be reasons for the adverse conditions for fetal development in MCTD.

Adult↗
PubMed1990

[Reflections on non-differentiated collagenosis or non-differentiated autoimmune syndrome].

The authors review the Non-Differentiated Collagenosis (NDC) described by Gyula Petrányi 29 years ago, and try to establish whether it is still necessary to maintain this clinical picture. Relying on data from special literature and on their own findings, the authors conclude that the maintenance of the NDC terminology is justified by the fact that poly-systemic autoimmune diseases take time to develop and that at certain stages of this development it is almost impossible to make a firm decision as to which clinical picture the process will lead to. The authors discuss the clinical and immunological features of NDC, the implications for the patients and things to be done in order to recognise the NDC disease process. They also emphasize a more frequent occurrence of the features of NDC than is generally stated, and that such patients need regular, competent and devoted medical attention.

Autoimmune Diseases↗
PubMed1989

[Clinical observations in mixed connective tissue disease].

The authors report their observations gathered by follow-up studies of many years duration on 67 patients suffering from mixed connective tissue disease. The period preceding the overt disease, its duration and its characteristic symptoms were examined. Concerning the severity of the clinical course, the authors distinguished between a less and a more severe form of the disease. Organ specific manifestations occurring in the two types of the disorder, the number of active phases per year, therapeutic tools and the prognosis were analysed. The authors assume that the more severe form of the disease may have some traits resembling those of systemic lupus erythematosus, but in the meantime, in the less severe type of the disease some features shared by rheumatic disorders can be found.

Adult↗
PubMed1989

Cytotoxic endothelial cell antibodies in mixed connective tissue disease.

We examined the presence of anti-endothelial cell antibodies in the sera of 44 patients with mixed connective tissue disease (MCTD). Warm antibodies against endothelial cells were found in 45.4% of patients; the presence of these antibodies was positively correlated with anti-monocyte antibodies (P less than 0.01) but not with anti-lymphocytic antibodies. Strong correlations were found between the presence of these antibodies and abnormalities of pulmonary ventilatory capacity, neurophysiologic and myocardial function. Anti-endothelial antibodies were related to the high rate of spontaneous abortion noted in female MCTD patients. The identification of the antigen identified by MCTD sera in endothelial cells would help in understanding disease manifestations.

Adult↗
PubMed1988

Methimazole blocks Graves' IgG but not interferon-gamma HLA-DR expression by thyroid cells.

We have expanded our early observation that a potent Graves' IgG preparation induces HLA-DR expression on thyroid cells, by showing that four randomly-selected Graves' IgG's were also capable of inducing DR antigens on thyroid cells. The effect of Graves' IgG was specific to thyroid cells, as it did not induce MHC Class II molecule expression on endothelial cells whereas interferon-gamma and immune complexes did so. The anti-thyroid drug methimazole was capable of rapidly reducing Graves' IgG-induced DR expression but to a much lesser extent than brought about by interferon-gamma. We conclude that Graves' IgG propagates thyroid-specific autoaggression by continued induction of DR antigens and than an important means whereby methimazole brings about remission is by reducing this induction.

Graves Disease↗