PubMed Health⌕ Search

Biomedical subjects

Margit Zeher

Publications and source records attributed to Margit Zeher.

At least 19 recordsLinked to original sources
PubMed2006

Clinical, serologic, and genetic profiles of patients with associated Sjögren's syndrome and systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS) can coexist in patients. The aim of this study was to investigate the clinical, laboratory, and serologic features of the association of the two diseases. Data from 56 patients having both SS and SLE were analyzed, with special emphasis on their clinical, laboratory, and serologic features. Data from 50 patients with SLE and 50 patients with SS served as control subjects. The mean age in SS-SLE group was lower than in patients with SS but higher than patients with SLE. When SLE and SS were associated, presence of rheumatoid factor was less frequent, whereas anti-Ro/SS-A, anti-La/SS-B, antinuclear, anti-DNA, and antiphospholipid autoantibodies appeared more often. Antiphospholipid syndrome, anemia, leucopenia, and lymphopenia were more frequent in the associated disease than in patients with SS alone. In SS-SLE patients, pulmonary, renal, skin, central nervous system involvement, and serositis occurred more often than in patients with SS alone. Thyroiditis, autoantibodies to Ro/SS-A, La/SS-B and ds-DNA was more frequent in the SS-SLE group than in patients with SLE. Genetic background of the patients did not differ significantly. In conclusion, characteristic clinical, laboratory and serologic features distinguish between the association of SS and SLE and the primary forms of these two diseases.

Adult↗
PubMed2006

Diastolic function of the heart in mixed connective tissue disease.

The authors examined the right and left ventricle functions in patients with mixed connective tissue disease (MCTD) by Doppler echocardiography. Of 51 patients, 20 had temporary pulmonary arterial hypertension in their case history. According to our knowledge, this is the first study examining the use of Doppler echocardiography and tissue Doppler technique in MCTD patients. Of 51 MCTD patients, 20 had pulmonary arterial hypertension (PAH) in the past 2 years. Diameters of the right and left ventricle, systolic and diastolic blood pressure were measured both in the 51 MCTD patients and in the 30 control subjects (mean age 54.8+/-6.2 years in the case of patients and 54.2+/-8.8 years in the case of control subjects). To estimate the global ventricle functions, the myocardial performance index--as described by Tei et al. (J Am Soc Echocardiogr 6:838-874, 1996)--was applied, which reflects the ratio of the sum of the isovolumetric contraction and relaxation time as compared to the ejection time. The 20 MCTD patients with PAH received cyclophosphamide therapy for 1 year beside the pulse corticosteroid (CS) therapy. In the case of MCTD patients without PAH, different treatments were used: 12 out of 31 patients were treated with sulfasalazine, 5 of whom received a combination of CS and methotrexate, and 14 took nonsteroid antiinflammatory drugs. In the case of the 51 MCTD patients (20 with PAH and 31 without PAH), diastolic function disorder of the left ventricle was detected; the diastolic Ee/Aa velocity quotient of the lateral mitral anulus was lower (p<0.01), and the mean deceleration time was longer (p<0.001) than that of the control group. The Tei index demonstrated the damage of the global ventricle function. The Tei index of the right ventricle indicated global failure of the right ventricle function in the case of MCTD patients complicated with PAH (Tei index 0.36+/-0.07 in MCTD with PAH and 0.28+/-0.04 in MCTD without PAH, p<0.001). The right ventricle function of MCTD patients without PAH was no different from that of the control group. In the case of patients with MCTD, signs of the disorder of the left ventricle diastolic function were observed. Our results suggest that the global impairment of the left ventricle function is the consequence of the disease itself and not the side effect of the treatment. In the case of MCTD patients complicated with PAH, the signs of the right ventricle function impairment proved to be permanent.

Adult↗
PubMed2006

Pulse cyclophosphamide therapy for inflammatory bowel disease.

AIM: To assess the efficacy of intravenous cyclophosphamide pulse therapy for refractory inflammatory bowel disease (IBD). METHODS: We included in our cohort eight patients with (moderate/severe) steroid refractory IBD (4 with ulcerative colitis and 4 with Crohn's disease). They all received 6 cycles of intravenous cyclophosphamide (800 mg) per month. RESULTS: Patients entered into remission after the second/third cyclophosphamide pulse. Disease activity decreased. There were no side effects and toxicity. All the patients went into long lasting remission. All Crohn's disease patients and 3 of 4 ulcerative colitis patients achieved complete remission. One patient with ulcerative colitis showed an impressive clinical response but did not enter into remission. For the maintenance, patients with Crohn's disease were treated with methotrexate (15 mg/wk) and patients with ulcerative colitis were treated with azathioprine (2.5 mg/kg body weight/d). CONCLUSION: Remission was maintained in all patients for 6 mo on the average. The drug was well tolerated. These findings suggest that aggressive immunosuppressive therapy may be useful in some refractory patients and further controlled study should be considered in order to fully evaluate this type of treatment as a potential therapy for IBD.

Adult↗
PubMed2005

[Association of systemic lupus erythematosus and Sjögren's syndrome].

INTRODUCTION: Systemic lupus erythematosus and Sjögren's syndrome are multisystemic autoimmune diseases which can be associated to each other. OBJECTIVE: To investigate if there are any distinct clinical, laboratory or serologic features due to the association of the two diseases that can influence the follow up of these patients. PATIENTS AND METHODS: The authors proved the association of these two autoimmune diseases in 56 patients, and these patients' clinical, laboratory and immunoserologic alterations. 50 patients with Sjögren's syndrome and 50 patients with systemic lupus erythematosus were used as control groups. RESULTS: Compared with Sjögren's syndrome alone, in the cases of the association of the diseases, rheumatoid factor was present less frequently, Ro/SS-A, La/SS-B and DNA antibodies were present more frequently, such as antiphospholipid autoantibodies and antiphospholipid syndrome. Anaemia, leukopenia and lymphopenia were detected more often and the patients were younger than in Sjögren's syndrome. Also, affection of the lung, kidney, skin, central nervous system and serous membranes are more common. The group with systemic lupus erythematosus differs in being older, having thyroiditis, Ro/SS-A, La/SS-B and DNA more frequently. CONCLUSION: Definitive clinical, laboratory and serological features make the difference between the association of the two diseases and the diseases observed alone.

Adult↗
PubMed2005

[Clinical experiences with cyclosporin A in patients with systemic lupus erythematosus].

INTRODUCTION: Cyclosporin A is a peptide composed of 11 amino acids. Its immune suppressive effect is attributed to the suppression of proliferation of T lymphocytes. Recently, besides immune suppression after organ transplantation it is used in the treatment of autoimmune disorders, such as systemic lupus erythematosus. AIMS: In the present work authors examined the frequency, effectiveness and safety of cyclosporin A in patients with systemic lupus erythematosus, regularly followed at the above mentioned department. RESULTS: Of the 450 lupus patients one male and 21 females received cyclosporin A for 12.4 months (3-96). Before cyclosporin A administration 16 patients received combined immune suppressive therapy; 68.2% azathioprine, 27.3% chloroquine, 18.2% methotrexate, while 3 patients were treated with steroid alone. High autoantibody titres clinical activity and the need for reduction of steroid dose were the mean indications. Furthermore, cyclosporin A was started because of leukopenia in 45.5%, polyarthritis in 36.4% or both in 27.3% of patients. Four patients started cyclosporin A to control skin eruptions. Thrombocytopenia, autoimmune hemolysis, Evans' syndrome and pancytopenia indicated this therapy in one patient for each. Clinical effectiveness was indicated by the reduction of mean daily methylprednisolone dose from 25.1 mg to 8.36 mg and also by the reduction of the disease activity index (SLE-DAI) from 9.47 to 2.47 within the first half year of the treatment. Therapy was well-tolerated. Only 3 patients observed hypertrichosis. Cyclosporin A was stopped in 3 cases due to infection and in 1 patient due to bad compliance. CONCLUSION: Present results suggest that cyclosporin A is an effective and safe treatment also in a certain group of lupus patients, especially with joint, skin and hematologic complications. Further long-term follow-up is required to evaluate late side-effects.

Adult↗
PubMed2005

Glucocorticosteroid therapy decreases CD14-expression and CD14-mediated LPS-binding and activation of monocytes in patients suffering from systemic lupus erythematosus.

In order to study the possible action of glucocorticosteroids (GCS) on the CD14/Toll like receptor mediated activation of monocytes the CD14-expression, CD14-mediated LPS binding and activation of these cells of patients suffering from Systemic Lupus Erythematosus receiving no, low dose or pulse steroid treatment was studied. The CD14-expression was determined on whole blood monocytes by flow cytometry, while the LPS-binding of an FITC-LPS preparate and the LPS-induced TNFalpha secretion were tested on isolated monocytes. The CD14-dependent and -independent LPS-binding and activation were evaluated with the help of a blocking anti-CD14 mAb. Our results showed that the CD14-expression, CD14-dependent LPS-binding and activation were significantly inhibited by the in vivo applied pulse steroid therapy. In contrast, the CD14-independent LPS-binding and activation were not altered by the GCS treatment. Our data provide further in vivo evidence for a possible new way of GCS therapy is able to initiate its anti-inflammatory action.

Adult↗
PubMed2005

[Interstitial lung disease in mixed connective tissue disease].

INTRODUCTION: The authors analyzed the incidence of interstitial lung disease in mixed connective tissue disease. They were seeking an answer to the following problems: the nature of the pathological course of mixed connective tissue disease complicated by and the therapy to be used in interstitial lung disease. PATIENTS AND METHODS: 179 patients were followed up during a period of 15.9 +/- 6.1 years. Interstitial lung disease was diagnosed using high resolution computed tomography. The diagnosis of interstitial lung disease was not obvious in 5 patients thus open lung biopsy was performed, which confirmed common interstitial pneumonitis. The patients were followed-up, and the data of computed tomography and respiratory function tests were detected 6 months, and then 4 years after the acute lung disease complicated by mixed connective tissue disease. RESULTS: Out of the 179 mixed connective tissue disease patients 96 (53.6%) had interstitial lung disease. The onset of interstitial lung disease was the most frequent in the 2-4 years of the disease. Four years after the first appearance of interstitial lung disease severe fibrosis was diagnosed in 24 patients (25%). A honey comb formation in the lung developed only in one patient. For the treatment of interstitial lung disease, corticosteroid treatment had to be combined with cyclophosphamide in 51 cases. In 4 patients (24%), pulmonary arterial hypertension evolved 2-4 years following interstitial lung disease. The high pulmonary arterial pressure decreased using pulsed corticosteroid treatment, cyclophosphamide, prostacyclin analogue, anticoagulants therapy and the 4 patients stay alive. The pulmonary arterial hypertension was caused by obliterative vasculopathy. CONCLUSION: Pulmonary involvement is found in more than half of the patients with mixed connective tissue disease. Early diagnosis of interstitial lung disease is possible by computed tomography. Interstitial lung disease can be treated by the combination of corticosteroids and cyclophosphamide. The authors were the first to detect the coexistence of interstitial lung disease and pulmonary arterial hypertension in mixed connective tissue disease. Subsequent respiratory alterations in these patient necessitate regular patient follow up.

Adult↗
PubMed2005

Association of systemic and thyroid autoimmune diseases.

OBJECTIVE: There are few large cohort studies available on the association of systemic and thyroid autoimmune diseases. In this study, we wished to determine the association of Hashimoto's thyroiditis (HT) and Graves' disease (GD) with systemic autoimmune diseases. METHODS: One thousand five hundred and seventeen patients with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc), mixed connective tissue disease (MCTD), Sjögren's syndrome (SS) and polymyositis/dermatomyositis (PM/DM) were included in the study. The HT and GD were diagnosed based on thorough clinical evaluation, imaging and fine-needle aspiration cytology (FNAC). The frequency of HT and GD in these diseases was assessed. In addition, 426 patients with HT or GD were assessed and the incidence of SLE, RA, SSc, MCTD, SS and PM/DM among these patients was determined. Prevalence ratios indicating the prevalences of GD or HT among our autoimmune patients in comparison to prevalences of GD or HT in the general population were calculated. RESULTS: Altogether 8.2% of systemic autoimmune patients had either HT or GD. MCTD and SS most frequently overlapped with autoimmune thyroid diseases (24 and 10%, respectively). HT was more common among MCTD, SS and RA patients (21, 7 and 6%, respectively) than GD (2.5, 3 and 1.6%, respectively). The prevalences of HT in SLE, RA, SSc, MCTD, SS and PM/DM were 90-, 160-, 220-, 556-, 176- and 69-fold higher than in the general population, respectively. The prevalences of GD in the same systemic diseases were 68-, 50-, 102-, 76-, 74- and 37-fold higher than in the general population, respectively. Among all thyroid patients, 30% had associated systemic disease. In particular, 51% of HT and only 16% of GD subjects had any of the systemic disorders. MCTD, SS, SLE, RA, SSc and PM/DM were all more common among HT patients (20, 17, 7, 4, 2 and 2%, respectively) than in GD individuals (2, 5, 5, 1, 2 and 1%, respectively). CONCLUSION: Systemic and thyroid autoimmune diseases often overlap with each other. HT and GD may be most common among MCTD, SSc and SS patients. On the other hand, these systemic diseases are often present in HT subjects. Therefore it is clinically important to screen patients with systemic autoimmune diseases for the co-existence of thyroid disorders.

Arthritis, Rheumatoid↗
PubMed2005

[Microscopic colitis].

AIM: Various case presentations in the literature discussed that frequent watery diarrheas introduce the clinical picture of microscopic colitis and intermittent or continuous diarrhea can remain. On the other hand, numerous cases never suffered from episodes of diarrhea but suffered from chronic constipation definitely. The authors present their first observations in Hungary. METHODS: A cohort of patients with independently confirmed typical histopathological changes was investigated. 53 patients with histologically proved MC (46 with CC, 7 with LC) were included. The existence of diarrhea or constipation and the co-existence of autoimmune diseases also investigated and all data were retrospectively analyzed. RESULTS: In contrast of the literature, about half of the patients had chronic constipation and cca. 50% of MC patients had autoimmune disease and the diagnosis of autoimmune disease was always prior to MC. CONCLUSIONS: Causes of inducing and amplifying the processes of "remodelling" the gut wall are thought to be unknown, but the results are: altered structure (and function?). All useful observations of this study may enrich the practice and can help these patients.

Adult↗
PubMed2005

Pulmonary arterial hypertension in mixed connective tissue disease: successful treatment with Iloprost.

This paper describes a 61-year-old woman who presented with mixed connective tissue disease, which was complicated by the development of pulmonary arterial hypertension (PAH). Her condition worsened rapidly, with development of haemopthysis, tachypnoe and cardiac arrest. Doppler echocardiography showed a high systolic pulmonary arterial pressure (98 mmHg), confirmed by the right heart catheterization. Vasculopathy of the pulmonary artery vessels was detected following open lung biopsy. No pulmonary embolism was found. Because of suspicion of flare of her underlying disease, which leads to PAH, immunosuppressive treatment was started with high doses of corticosteroid and cyclophosphamide, in combination with the prostacyclin analogue, Iloprost, and low molecular weight heparin. The therapy resulted in slow recovery over 6 weeks, with control echocardiography showing normalization of the high pulmonary pressure, and the patient being capable of returning to everyday activities.

Autoantibodies↗
PubMed2005

Polymyalgia rheumatica in primary Sjögren's syndrome.

The aim of the study was to describe the clinical and laboratory aspects of primary Sjögren's syndrome (pSS) associated with polymyalgia rheumatica (PMR). The retrospective study compares the clinical and laboratory aspects of patients with pSS associated with PMR on a relatively large cohort of patients (n=16) and pSS patients without PMR (n=531). The prevalence of PMR among pSS patients was 3%, while in the average population, the prevalence of PMR is only 0.75%. PMR developed 8.7 years after the diagnosis of pSS in the older female pSS population (over 50 years of age), and in those with only glandular features. Interestingly the pSS/PMR patients had hypo gammaglobuline levels, while in the pSS patient group hypergammaglobulinaemia presented. Furthermore, positive ANA serology was more frequent among pSS/PMR patients. Since the clinical management of pSS/PMR is different from pSS, a better understanding of this clinical entity is essential.

Age Factors↗
PubMed2005

Microscopic colitis: a retrospective study of clinical presentation in 53 patients.

AIM: To evaluate the relationship between symptoms and microscopic colitis (MC) subtypes: to test whether collagenous colitis (CC) and/or lymphocytic colitis (LC) might be related to both constipation and diarrhea. METHODS: A cohort of patients with independently confirmed typical histopathological changes was investigated. Fifty-three patients with histologically proved MC (46 with CC, 7 with LC) were included. The existence of diarrhea or constipation and the co-existence of autoimmune diseases were also investigated and all data were retrospectively analyzed. RESULTS: Twenty-three (43.39%) of MC patients had chronic constipation (20 in CC, 3 in LC patients). Twenty-four (45.28%) of MC patients had autoimmune disease and the diagnosis of autoimmune disease was always prior to MC. Sjögren's syndrome was associated only with the constipation subgroup. CONCLUSION: The Janus face of MC resembles the subgroups of irritable bowel syndrome. The co-existence of autoimmune diseases and MC is confirmed in both the constipation and diarrhea subgroups.

Adult↗
PubMed2005

[Is the primary antiphospholipid syndrome a forerunner of SLE?].

INTRODUCTION: Antiphospholipid syndrome is a multi-organ autoimmune disorder, characterized by arterial and venous thrombotic events, and a well-defined group of recurrent foetal wasteage due to pathologic antibodies against phospholipids and protein co-factors. Antiphospholipid antibodies can be formed in primary antiphospholipid syndrome, but also in other conditions, most often in systemic lupus erythematosus. This may modify the outcome of lupus increasing the risk for thrombotic complications. Less is known about the outcome in primary antiphospholipid syndrome, whether it may precede the development of systemic lupus. AIMS: Authors hereby describe the case of four patients with primary antiphospholipid syndrome in whom the disease progressed to systemic lupus erythematosus. RESULTS: Lupus followed the primary antiphospholipid syndrome within around a three-year period. It was indicated by the appearance of different antinuclear autoantibodies and clinical complications, such as polyarthritis, nephritis and hematologic disturbances. All of the patients presented cerebrovascular accident as the thrombotic manifestation. All but one, were around forty years old, and had a milder form of lupus. Symptoms of the antiphospholipid syndrome determined the outcome. On the other hand, a typical lupus developed in the youngest patient. CONCLUSIONS: According to present cases antiphospholipid syndrome may be considered as the initiative phase of SLE, but APS being a separate entity also may associate to lupus. Present observations indicate the importance of follow-up the patients with APS by immunologic respect. Future prospective, multi-centre studies with larger number of cases are needed to provide further evidence on the fact that patients with APS may acquire other autoimmune disorder.

Adult↗
PubMed2005

Homozygosity for the 168His variant of the minor histocompatibility antigen HA-1 is associated with reduced risk of primary Sjögren's syndrome.

The genes for the human ATP-binding cassette (ABC) transporter ABCA7 and the minor histocompatibility antigen HA-1 are juxtaposed in close proximity on chromosome 19p13.3. The multispan transmembrane protein ABCA7 contains an extracellular domain that is recognized by antisera from patients with Sjögren's syndrome ("Sjögren-epitope"). Recent work from our laboratory demonstrating the involvement of ABCA7 in cellular ceramide and phosphatidylserine export suggests a role for this transporter in programmed cell death. In HA-1, a protein of unknown function, a His/Arg polymorphism (His168Arg), which constitutes the immunologic target for HA-1-specific cytotoxic T cells, has been causatively linked to graft-versus-host disease after allogeneic stem cell transplantation. Because these findings suggest a potential implication of ABCA7 and HA-1 in immune processes, we tested the hypothesis that allelic variants in both genes are associated with autoimmune disorders. We identified a total of 31 exonic single-nucleotide polymorphisms (SNP) in the ABCA7/HA-1 gene complex, nine of which represent non-synonymous nucleotide alterations. Genotypes of ABCA7 and HA-1 SNP were determined in three distinct Caucasian populations of patients with primary Sjögren's syndrome and ethnically matched controls. Comparison of allele frequencies between these groups revealed that the incidence of the HA-1 168His allele is significantly lower in Sjögren's syndrome patients than in controls (p<0.003). In contrast, the frequencies of all ABCA7 allelic variants and additional HA-1 polymorphisms were similar in patients and controls. In cohorts of patients with systemic lupus erythematosus, rheumatoid arthritis and multiple sclerosis, no significant differences in the frequencies of ABCA7 and HA-1 allelic variants were observed relative to controls. Our results suggest that the HA-1 168His variant is associated with reduced susceptibility to primary Sjögren's syndrome.

ATP-Binding Cassette Transporters↗
PubMed2005

High prevalence of oesophageal involvement in patients with undifferentiated connective tissue disease using radionuclide oesophageal transit scintigraphy.

AIM: To look for the frequency of oesophageal dysfunction using radionuclide oesophageal transit scintigraphy in 145 patients with undifferentiated connective tissue disease (UCTD); to seek the correlation between the clinical/laboratory data and scintigraphic alterations; and to determine predictive value of radionuclide oesophageal transit scintigraphy for evolution to established connective tissue disease (CTD). METHOD: One hundred and forty-five patients with UCTD were examined by 99mTc-DTPA oesophageal transit scintigraphy. The intraoesophageal transport of the radiopharmaceutical was followed and imaged by a gamma camera, a series of 128 x 128 images were stored and evaluated. The correlation between the scintigraphic data and clinical and laboratory parameters was analysed statistically. RESULTS: Unequivocally positive scintigraphy, indicative of motor abnormality was found in 46% of patients (66), 71% (47) of whom were totally asymptomatic. Significant correlation was found between the presence and severity of scintigraphic alterations and antinuclear antibodies, the anti-beta2GPI, IgM, IgG, the aCL antibody positivity, and the skin symptoms. Scintigraphic positivity was significantly more frequent in patients evolving to definitive CTD (P = 0.0178), and abnormal scan predisposed to transition into the definitive CTD (odds ratio, 2.292; CI, 1.610-4.525). Its cumulative positive predictive value was found to be 43% and cumulative negative predictive value 73% with regard to the development of a definitive CTD. CONCLUSION: Our results show that scintigraphic alterations together with clinical and laboratory alterations can help the clinician in the prediction of final outcome.

Adolescent↗
PubMed2005

Sjögren syndrome associated with hepatitis C virus: a multicenter analysis of 137 cases.

To define the clinical and immunologic pattern of expression of Sjögren syndrome (SS) associated with chronic hepatitis C virus (HCV) infection, we conducted a multicenter study aiming to collect a large number of patients with SS and HCV infection. Inclusion criteria were the fulfillment of at least 4 of the classification criteria for SS proposed by the European Community Study Group and repeated positive HCV serology, confirmed by recombinant immunoblot assay and/or detection of serum HCV-RNA by polymerase chain reaction. One hundred thirty-seven patients were included (104 female and 33 male; mean age, 65 yr). Seventy-nine (58%) patients presented a systemic process with diverse extraglandular manifestations, with articular involvement (44%), vasculitis (20%), and neuropathy (16%) being the most frequent features observed. The main immunologic features were antinuclear antibodies (65%), hypocomplementemia (51%), and cryoglobulinemia (50%). Cryoglobulins were associated with a higher frequency of cutaneous vasculitis, rheumatoid factor, and hypocomplementemia. Thirty-two (23%) patients had positive anti-Ro/SS-A and/or anti-La/SS-B antibodies; these patients were predominantly women and had a higher prevalence of some extraglandular features and a lower frequency of liver involvement. Nineteen (14%) patients developed neoplasia, with hematologic neoplasia (8 cases) and hepatocellular carcinoma (6 cases) being the most frequent types. Eighty-five percent of SS-HCV patients also fulfilled the recently proposed 2002 classification criteria for SS. In conclusion, HCV-associated SS is indistinguishable in most cases from the primary form using the most recent set of classification criteria. Chronic HCV infection should be considered an exclusion criterion for the classification of primary SS, not because it mimics primary SS, but because the virus may be implicated in the development of SS in a specific subset of patients. We propose the term "SS secondary to HCV" when these patients fulfill the 2002 classification criteria for SS.

Adult↗