PubMed Health⌕ Search

Biomedical subjects

L Czirják

Publications and source records attributed to L Czirják.

At least 19 recordsLinked to original sources
PubMed2006

Lack of association between three vascular endothelial growth factor gene polymorphisms and systemic sclerosis: results from a multicenter EUSTAR study of European Caucasian patients.

INTRODUCTION: Systemic sclerosis (SSc) is characterised by disturbed vessel morphology and an overproduction of vascular endothelial growth factor (VEGF). The VEGF gene located on chromosome 6p21.3 has several polymorphisms. OBJECTIVE: To test the hypothesis that disturbed angiogenesis may be related to the genetic background of the VEGF gene. MATERIALS AND METHODS: EUSTAR centres included European Caucasian patients with SSc and matched controls with osteoarthritis. The VEGF gene was genotyped by polymerase chain reaction, followed by restriction enzyme analysis. The 634 C/T and 936 C/G mutations and an 18-base pair insertion/deletion at -2549 of the VEGF promoter region were tested. RESULTS: 416 patients with SSc and 249 controls were included in the study population. Of the patients with SSc, 42% had a diffuse cutaneous subtype, 16% had increased pulmonary arterial pressure and 61% had decreased carbon monoxide diffusion capacity. The genotype frequencies in the patients with SSc and in controls were in Hardy-Weinberg equilibrium. The allele and genotype frequencies of the polymorphisms did not differ between patients with SSc and controls. No association was found between these polymorphisms and disease phenotypes. CONCLUSION: This study shows that there is no association between the three selected functional VEGF polymorphisms and SSc.

Adult↗
PubMed2004

Practical approach to the therapy of systemic sclerosis.

Systemic sclerosis (SSc) is characterised by vascular abnormalities, fibrosis, inflammatory changes, and atrophy affecting the skin and several internal organs. The course of the disease is highly variable from patient to patient, and the different organ manifestations may show a different stage of activity and damage. More extensive skin involvement coincides with more severe internal organ manifestation(s) resulting in an unfavourable outcome of disease. Subset classification and organ system involvements should be carefully determined before introducing the therapy. Prognostic evaluation, including the staging of disease severity, damage, activity and the definition of longitudinal disease behaviour of the organ manifestations (by serial investigations) is also crucial. Theoretically the treatment should focus on both the early reduction of inflammatory phenomena and influence of vascular symptoms to prevent the development of tissue fibrosis and atrophy. Major internal organ manifestations including gastro-oesophageal reflux, scleroderma renal involvement, different cardio-respiratory disorders should also be carefully looked for, and early signs of internal organ involvements should be recognized and treated. There is no disease modifying drug therapy of proven efficacy in SSc but an individualized, well designed therapy may be remarkably efficient in terms of improving the prognosis and quality of life of patients. Education of patients is also compelling.

Antirheumatic Agents↗
PubMed2004

Nailfold digital capillaroscopy in 447 patients with connective tissue disease and Raynaud's disease.

The presence of megacapillaries and a decreased capillary density are the hallmarks of the scleroderma capillary pattern, which can be detected by nailfold capillarmicroscopy. One hundred and eighty-six patients with Raynaud's phenomenon, 65 cases with undifferentiated connective tissue disease (UCTD), 47 patients with systemic lupus erythematosus (SLE), 26 patients with dermato/polymyositis, 14 with rheumatoid arthritis, seven cases with primary Sjögren's syndrome and 102 patients with systemic sclerosis (SSc) were investigated. Of the 16 patients with diffuse cutaneous SSc and the 86 limited cutaneous SSc cases, 14 (87.5%) and 53 (61.6%) showed the scleroderma capillary pattern, respectively. Nine of the 65 (13.8%) cases with UCTD and 24 of the 186 (12.9%) cases with Raynaud's phenomenon also exhibited the same pattern. Four of the 47 (8.5%) with SLE and seven of the 26 (26.9%) with dermato/polymyositis, and no patients with rheumatoid arthritis or Sjögren's syndrome, exhibited the scleroderma capillary pattern. The conclusion is that the scleroderma capillary pattern is often present in SSc and dermato/polymyositis. Furthermore, patients with Raynaud's phenomenon and UCTD may also occasionally exhibit this pattern. Therefore, capillarmicroscopy seems to be a useful tool for the early selection of those patients who are potential candidates for developing scleroderma spectrum disorders.

Adult↗
PubMed2002

Exposure to solvents in female patients with scleroderma.

The role of exposure to solvents was investigated in female patients with connective tissue disease and Raynaud's phenomenon using a questionnaire. Sixteen out of the 63 patients with systemic sclerosis had been exposed to solvents. A borderline significance was demonstrated compared to matched female controls (P < 0.05). Fourteen out of the 66 patients with undifferentiated connective tissue disease, 18/86 of patients with Raynaud's phenomenon, 6/45 with systemic lupus erythematosus, 1/16 with dermatopolymyositis, 1/15 with rheumatoid arthritis and 0/13 with primary Sjögren's syndrome had been exposed to solvents. None of these groups of patients showed a statistical significance compared to matched controls. Our present findings indicate that, at least in certain areas of the world, exposure to solvents may be a provoking factor in female scleroderma, but it must be emphasised that only a borderline significance was found between the scleroderma patients and controls. A large multicenter study seems to be required to clarify the importance of solvents as provoking factors of scleroderma. Furthermore, exposure to solvents does not seem to be a provoking factor among females for the other connective tissue diseases.

Adult↗
PubMed2002

Fatal scleroderma renal crisis caused by gastrointestinal bleeding in a patient with scleroderma, Sjögren's syndrome and primary biliary cirrhosis overlap.

A 57-year-old female patient presented with a long history of overlapping autoimmune disease, including limited cutaneous systemic sclerosis, Sjögren's syndrome and primary biliary cirrhosis. Another unusual finding was that the mild skin involvement (limited cutaneous systemic sclerosis, subcutaneous calcinosis) was combined with serious internal organ involvement, including honeycombing and finally scleroderma renal crisis. The most important finding was, that two decades later she developed severe telangiectasia of the gastrointestinal and urinary tract. Furthermore, a specific type of vascular malformation, i.e. gastric watermelon stomach was also found. The chronic gastrointestinal bleeding primarily derived from a watermelon stomach caused protein overload, which provoked the onset of the scleroderma renal crisis that finally led to the patient's death.

Acute Kidney Injury↗
PubMed2001

[Barrett esophagus in systemic sclerosis].

Systemic sclerosis is a generalized systemic autoimmune disorder frequently involving the oesophagus causing reflux oesophagitis in the majority of patients. Barrett's oesophagus is one of the most serious complication of the reflux oesophagitis, in which the normal oesophageal squamous epithelium is partially replaced by metaplastic columnar epithelium. The clinical significance of Barrett's oesophagus is the significantly increased risk of developing the adenocarcinoma. In our paper, the authors present 5 cases with Barrett's oesophagus among their 52 examined patients with scleroderma.

Adult↗
PubMed2001

Levels of antibodies against C1q and 60 kDa family of heat shock proteins in the sera of patients with various autoimmune diseases.

Previously a strong positive correlation was found between antibodies to C1q (C1qAb) and antibodies against human heat shock protein (hsp60) and mycobacterial hsp65 in HIV infected patients. Here the levels of these antibodies were measured in the sera of patients with different autoimmune diseases (122 systemic lupus erythematosus (SLE), 55 systemic sclerosis, 33 undifferentiated connective tissue disease (UCTD), 27 primary Raynaud syndrome, 21 rheumatoid arthritis (RA), 14 polymyositis/dermatomyositis (PM/DM), and 192 healthy blood donors. The prevalence of IgG C1qAb was found to be high (P<0.0001 as compared to the healthy controls) only in the SLE group. The levels of the anti-hsp60 (P=0.0094) and anti-hsp65 (P=0.0108) antibodies were high only in the UCTD patients. No correlation was found between the C1qAb and anti-hsp antibodies in any group except a significant (P=0.011) positive correlation between C1qAb and hsp65 antibodies in the patients with UCTD. These findings indicate that the autoantibodies against C1q are heterogeneous: in different diseases different types of C1qAb may dominate.

Adult↗
PubMed2001

Alveolitis may persist during treatment that sufficiently controls muscle inflammation in myositis.

Eight patients with dermato- and polymyositis (DM/PM) and two further cases with sclerodermamyositis overlap syndrome were investigated. These patients showed signs of lung manifestation by noninvasive methods. Bronchoalveolar lavage (BAL) was performed to detect alveolitis. Four of the eight DM/PM patients showed elevated neutrophil counts. All of these and one additional case had increased lymphocyte counts. Five of the DM/PM patients showed higher total cell numbers than five healthy controls. One of the cases with scleroderma-myositis overlap syndrome also developed lymphocyte alveolitis. We conclude that signs of alveolitis are often present in patients with myositis, even though the myositis was adequately controlled by corticosteroid therapy and, in four cases, with corticosteroid plus azathioprine. The need for further follow-up studies to determine the effectiveness of intensified corticosteroid/cytostatic treatment in these patients is emphasised.

Adult↗
PubMed2001

Subsets in psoriatic arthritis formed by cluster analysis.

The aim of the study was to create subgroups among psoriatic arthritis patients on the basis of dermatological features, clinical pattern of arthritis, and laboratory, immunological and radiological findings. Data on 100 patients were expressed in a standardised form and entered into hierarchical cluster analysis according to Ward's method. Seven subgroups were created. Fifty-six patients with mild psoriasis were sorted into a 'polyarticular group'. Two 'RA-like groups' were formed, differing from each other serologically and in axial involvement. In an 'oligoarticular group' (18 patients) serious skin disease and female gender predominancy were found to be characteristic. Eight patients with polyarticular arthritis were assigned to an 'erythrodermal group', in which polyarticular arthritis, mutilating, severe arthritis and a history of erythroderma were characteristic. Close to this group on the dendrogram eight women were sorted into a 'distal form'. Sausage fingers were frequent, and nail dystrophy was present in every case. In a 'pustular group' (three patients) the different type of skin involvement was considered and nail dystrophy was common. In the newly created subgroups not only the arthritic status, but also the type of the skin disease, played a determining role.

Adult↗
PubMed2001

Histiocytic necrotizing lymphadenitis preceding systemic lupus erythematosus.

Necrotizing histiocytic lymphadenopathy (NHL) is a rarely observed clinical entity that is occasionally associated with systemic lupus erythematosus (SLE). The histological features of the condition have been considered to be indistinguishable from those of lymphadenitis in subjects with SLE, and the clinical symptoms of the two disorders share common features. This report presents the case history of a subject who developed SLE with central nervous system involvement 3 years following onset of Kikuchi's disease (histiocytic necrotizing lymphadenitis). Repeated lymph node biopsies confirmed the diagnosis in relation to the clinical progression. A review of the literature on this topic is also presented.

Adrenal Cortex Hormones↗
PubMed2001

Increased urinary pyridinoline cross-link compounds of collagen in patients with systemic sclerosis and Raynaud's phenomenon.

OBJECTIVE: To study concentration changes in collagen degradation markers in patients with diffuse and limited cutaneous systemic sclerosis and patients with scleroderma-related diseases. METHODS: Pyridinoline cross-link compounds were analysed in urine samples using high-performance liquid chromatography. Samples were analysed for pyridinoline (Pyr), deoxypyridinoline (Dpyr) and soft-tissue pyridinoline (stPyr) in patients with diffuse cutaneous systemic sclerosis (dcSSc, n=23) and limited cutaneous systemic sclerosis (lcSSc, n=48) and in patients with scleroderma-related diseases such as primary Raynaud's phenomenon (pRP, n=16) and secondary Raynaud's phenomenon (sRP, n=14). Healthy controls (n=18) and patients with post-menopausal osteoporosis (OP, n=35) were also investigated. RESULTS: Urinary Pyr, Dpyr and stPyr concentrations were significantly higher in patients with Raynaud's phenomenon and systemic sclerosis than in healthy controls. The highest concentrations (two to three times greater than in healthy controls) were found in patients with dcSSc. The stPyr concentration was significantly higher in patients with dcSSc than in those with lcSSc, sRP and pRP. No significant difference in stPyr concentration was found between the healthy controls and the OP group, suggesting that stPyr is derived from soft tissues rather than bone. The extent and severity of skin involvement, measured as a skin score, significantly correlated with the concentrations of stPyr and Pyr, whereas no such correlation was found for Dpyr. CONCLUSIONS: Increased urinary concentrations of piridinoline cross-links reflect alterations in collagen turnover in both Raynaud's phenomenon and systemic sclerosis. The close correlation between stPyr concentration and the extent of skin involvement in systemic sclerosis suggests that this parameter may be useful in monitoring ongoing fibrosis in this disease.

Amino Acids↗
PubMed2001

European multicentre study to define disease activity criteria for systemic sclerosis. I. Clinical and epidemiological features of 290 patients from 19 centres.

OBJECTIVE: To investigate the existence of differences among European referral centres for systemic sclerosis (SSc) in the pattern of attendance and referral and in the clinical and therapeutical approaches. METHODS: In 1995 the European Scleroderma Study Group initiated a multicentre prospective one year study whose aim was to define the disease activity criteria in SSc. During the study period each participating European centre was asked to enroll consecutive patients satisfying American College of Rheumatology criteria for SSc and to fill out for each of them a standardised clinical chart. Patients from various centres were compared and differences in epidemiological, clinical, and therapeutical aspects were analysed. RESULTS: Nineteen different medical research centres consecutively recruited 290 patients. The patients could be divided into two subgroups: 173 with the limited (lSSc) and 117 with the diffuse (dSSc) form of the disease. The clinical and serological findings for the series of 290 patients seemed to be similar to data previously reported. However, when the data were analysed to elicit any differences between the participating centres, a high degree of variability emerged, in both epidemiological and clinical features and in the diagnostic and therapeutic approaches to the disease. CONCLUSIONS: The clinical approach to SSc, not only in different countries but also in different centres within the same country, is not yet standardised. To overcome this problem, it will be necessary for the scientific community to draw up a standardised procedure for the management of patients with SSc. This would provide a common research tool for different centres engaged in research on this complex disease.

Adolescent↗
PubMed2001

European multicentre study to define disease activity criteria for systemic sclerosis. II. Identification of disease activity variables and development of preliminary activity indexes.

OBJECTIVE: To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use. METHODS: Investigators from 19 European centres completed a standardised clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0-10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity ("inactive to moderately active" or "active to very active" disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed. RESULTS: A total of 290 patients with SSc (117 with diffuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including Delta-factors-that is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity: (a) Delta-cardiopulmonary (4.0), Delta-skin (3.0), Delta-vascular (2.0), and Delta-articular/muscular (1.0) for patients with dSSc; (b) Delta-skin (2.5), erythrocyte sedimentation rate (ESR) >30 mm/1st h (2.5), Delta-cardiopulmonary (1.5), Delta-vascular (1.0), arthritis (1.0), hypocomplementaemia (1.0), and scleredema (0.5) for lSSc; (c) Delta-cardiopulmonary (2.0), Delta-skin (2.0), ESR >30 mm/1st h (1.5), total skin score >20 (1.0), hypocomplementaemia (1.0), scleredema (0.5), digital necrosis (0.5), Delta-vascular (0.5), arthritis (0.5), TLCO <80% (0.5) for all patients with SSc. The three indexes were validated by the jackknife technique. Finally, receiver operating characteristic curves were constructed in order to define the value of the index with the best discriminant capacity for "active to very active" patients. CONCLUSIONS: Three feasible, reliable, and valid preliminary indices to define disease activity in SSc were constructed.

Adolescent↗
PubMed2001

Novel approaches in the treatment of lupus nephritis.

In systemic lupus erythematosus hyperactive helper T-cells drive polyclonal B-cell activation and secretion of pathogenic auto-antibodies. The auto-antibodies form immune complexes with their respective auto-antigens, which in turn deposit in sites such as the kidney and initiate a destructive inflammatory reaction. Lupus nephritis can be managed successfully in the majority of cases; however, the most widely used immunosuppressive therapies, notably corticosteroids and cyclophosphamide are non-specific and are associated with substantial toxicities. Novel treatments for lupus nephritis have to be at least as effective and less toxic than existing therapies. The ultimate aim is to develop treatments that target specific steps in the disease process. Novel therapeutic strategies in the short-term more likely will focus on refining regimens of drugs that are already in use (mycophenolate mofetil, adenosine analogues) and combinations of existing chemotherapeutic agents, as well as attempts to achieve immunological reconstitution using immunoablative chemotherapy with or without haematopoietic stem cell rescue. Several new agents targeting specific steps in the pathogenesis of lupus are in various phases of clinical development. Interrupting the interactions between T-lymphocytes and other cells by blocking co-stimulatory molecules, such as CD40 ligand or CTLA4-Ig, may interfere with the early steps of pathogenesis. Blocking IL-10 may decrease auto-antibody production and help normalise T-cell function. Treating patients with DNase or interfering with the complement cascade by blocking C5, or neutralising pathogenic antibodies by administering specific binding peptides or inducing specific anti-idiotype antibodies may prevent immune complex formation and/or deposition.

Animals↗
PubMed2001

[Cephalometric aspects of progressive systemic sclerosis--a case report].

The Progressive Systemic Sclerosos (PSS) is an autoimmune disease which affects mainly the middle age groups and is manifested in the fibrosis of different internal organs. The disease is important for the dentist because of the restricted mouth opening leading to the difficulties in oral hygiene and the routine dental treatment. The oral involvement in Progressive Systemic Sclerosis has already been studied, and a few alteration and radiological changes were detected. However cephalometric analysis of this patient group has not been performed up to now. In our study the oral clinical and radiological signs of the disease has been described in a patient with PSS. Cephalometric analysis (according to Ricketts, modified and to Holdoway) has been used to show the radiological anomalies of her jaws and soft tissues. The most important data of the jaws were registered in changes of SNB and AB angles. In soft tissues the most important cephalometrical changes were in the perioral region probably because of the sclerosis. This method (cephalometric method) would be a new possibility to describe objectively the clinical signs (mainly the changes in soft tissues) of patients with PSS.

Cephalometry↗
PubMed1999

Investigation of the alveolar macrophages and T lymphocytes in 15 patients with systemic sclerosis.

The cell distribution and function of alveolar macrophages and T lymphocytes were investigated in the bronchoalveolar lavage (BAL) of 15 patients with systemic sclerosis (SSc). In alveolar macrophages, both spontaneous and PMA-stimulated TNF-alpha production were increased in SSc. PMA-induced IL-6 production was also elevated. Spontaneous IL-6 excretion of scleroderma alveolar macrophages was similar to the controls. Yeast and C3b-coated yeast (opsonised yeast) phagocytosis, chemotaxis and Fc receptor activity of alveolar macrophages were normal. The proportion of CD3, CD4 and CD8 T-lymphocyte subsets in the BAL was similar to the control values. The lymphocyte blast transformation index of the non-adherent cells deriving from the BAL fluid was markedly decreased.

Adult↗
PubMed1999

Watermelon-stomach as a cause of chronic iron deficiency anemia in a patient with systemic sclerosis.

Watermelon-stomach is a rare cause of gastrointestinal bleeding. There has been an increasing number of reports on the association of this lesion with diseases of the scleroderma group, causing chronic, sometimes severe gastrointestinal blood loss. The present report presents the case of a 75-year-old female with limited cutaneous systemic sclerosis and watermelon-stomach, which was the cause of her long-standing sideropenic anemia.

Aged↗