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Elzbieta Waszczykowska

Publications and source records attributed to Elzbieta Waszczykowska.

8 recordsLinked to original sources

Disturbances of the expression of metalloproteinases and their tissue inhibitors cause destruction of the basement membrane in pemphigoid.

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease which pathogenesis is associated with destruction of the basement membrane components and the anchoring fibers. The binding of autoantibodies to antigens localized in the basement membrane of the epidermis activates a series of immunological and enzymatic phenomena that lead to blister formation. There are some data that MMPs are involved in the development of skin lesions in BP, however their exact role in this process is not fully understood. We aimed to investigate whether MMPs and their inhibitors (TIMPs), assessed by their tissue expression, are involved in the pathogenesis of BP. The localization and expression of collagenase (MMP1), gelatinase (MMP2), 92 kD gelatinase (MMP9) and stromelysin 2 (MMP10) and TIMP1, 2, 3 were examined by immunohistochemistry in skin biopsies as well as in normal human skin specimens. The study included 21 patients with BP at an active stage of the disease. The MMPs and TIMPs serum levels were measured by ELISA method. Expression of MMP1, MMP2, MMP9 and MMP10 was observed either in the whole epidermis or in the basal keratinocytes. Most of the enzymes examined, apart from TIMP3, were detected in dermal part of the blister. Expression of the majority of the enzymes examined was observed in blister fluid however, the most intense signal was noted for MMP10. In cellular infiltrate we found expression of all the MMPs and TIMPs, the most distinct for MMP1, MMP2, MMP10 and for TIMP2. In all biopsies obtained from healthy volunteers only single basal keratinocytes gave positive, weak signal for the examined proteins. The MMPs and TIMPs serum levels in the control group were normal while in some cases of BP patients they were increased. Based on the results we conclude that imbalance between these enzymes really occurs in BP and it is likely to take important part in the pathogenesis of the disease.

Aged↗

The imbalance between metalloproteinases and their tissue inhibitors is involved in the pathogenesis of dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is a subepidermal autoimmune disease characterized by skin and intestinal lesions consistent with coeliac disease. There are also some data that metalloproteinases (MMPs) are involved in the development of skin lesions in DH, however their exact role in this process is not fully understood. The aim of the study was to investigate whether MMPs and their inhibitors are involved in pathogenesis of DH. Skin biopsies were taken from 13 patients with active DH and from 10 healthy subjects. The localization and expression of MMPs and TIMPs were examined by immunohistochemistry. MMPs expression was detected in basal keratinocytes and in the whole epidermis in all of the DH subjects. Neutrophils in microabscesses and in blister fluid were also positive for MMPs. Expression of TIMPs was moderate or weak in all examined biopsies. Our results allow us to conclude that imbalance between these enzymes takes an important role in the pathogenesis of DH.

Adolescent↗

Correlation of endostatin and tissue inhibitor of metalloproteinases 2 (TIMP2) serum levels with cardiovascular involvement in systemic sclerosis patients.

Fibrosis of oesophagus, lungs, heart, and kidney in the course of systemic sclerosis (SSc) may lead to dysfunction of the above organs or even patients death. Recent studies point out the role of angiogenesis and fibrosis disturbances in the pathogenesis of SSc. Heart fibrosis is one of the most important prognostic factors in SSc patients. So, the aim of our study was to examine cardiovascular dysfunction in SSc patients and its correlation with serum levels of vascular endothelial growth factor (VEGF), endostatin, and tissue inhibitor of metalloproteinase 2 (TIMP2). The study group comprised 34 patients (19 with limited scleroderma (lSSc) and 15 with diffuse scleroderma (dSSc)). The control group consisted of 20 healthy persons, age and sex matched. Internal organ involvement was assessed on the basis of specialist procedures. Serum VEGF, endostatin, and TIMP2 levels were evaluated by ELISA. We found cardiovascular changes in 15 patients with SSc (8 with lSSc and 7 with dSSc). The observed symptoms were of different characters and also coexisted with each other. Higher endostatin serum levels in all systemic sclerosis patients in comparison to the control group were demonstrated (P < .05). Also higher serum levels of endostatin and TIMP2 were observed in patients with cardiovascular changes in comparison to the patients without such changes (P < .05). The obtained results support the notion that angiogenesis and fibrosis disturbances may play an important role in SSc. Evaluation of endostatin and TIMP2 serum levels seems to be one of the noninvasive, helpful examinations of heart involvement in the course of systemic sclerosis.

Adolescent↗

Evaluation of caspase 1 and sFas serum levels in patients with systemic sclerosis: correlation with lung dysfunction, joint and bone involvement.

Recent studies point out at the role of apoptosis disturbances in the development of systemic sclerosis(SSc). The aim of our study was to examine caspase 1 and sFas serum levels in scleroderma patients and correlate the obtained results with skin involvement and internal organ changes. We studied 29 patients (14 with limited and 15 with diffuse SSc). The extension of skin involvement was measured using Total Skin Score (TSS). Internal organ involvement was assessed by specialist procedures. Serum caspase 1 and sFas levels were measured by enzyme-linked immunosorbent assay. We found correlation between sFas serum level and duration of Raynaud's phenomenon and TSS; caspase 1 serum level correlated only with TSS. Correlations between caspase 1 and lung dysfunction and sFas levels with joint and bone involvement in SSc patients were also observed. The obtained results revealed that disturbances of apoptosis might play a role in SSc pathogenesis. Caspase 1 and sFas serum levels correlate with the skin involvement severity, lung dysfunction, joint and bone changes.

Adolescent↗

Detection of pemphigus autoantibodies by IIF and ELISA tests in patients with pemphigus vulgaris and foliaceus and in healthy relatives.

BACKGROUND: Pemphigus is a life-threatening, autoimmune blistering disease, mediated by IgG autoantibodies. The aim of our study was to assess the usefulness of a new enzyme-linked immunosorbent assay (ELISA) in detecting circulating pemphigus autoantibodies, and to compare its sensitivity and specificity with the indirect immunofluorescence (IIF) test. We also established the frequency of occurrence of pemphigus autoantibodies in relatives of our patients. MATERIAL/METHODS: IIF and ELISA tests were performed in 24 patients with pemphigus vulgaris, 13 with pemphigus foliaceus, 56 healthy relatives, and 50 controls, selected according to sex and age. RESULTS: The obtained results revealed high specificity and sensitivity of ELISA, comparable to the IIF test, especially in patients who were in the active stage of the disease. We also showed that the profile of anti-Dsg 1 and/or anti-Dsg 3 autoantibodies is associated with the clinical variant of pemphigus. The frequency of occurrence of pemphigus autoantibodies in the relatives (24/55) performed by IIF was significantly higher (p<0.001) than in the controls (0/50). The same antibodies detected by ELISA (11/56) were less frequent. CONCLUSIONS: In clinically doubtful cases, in which autoantibodies titres by IIF are equal on both substrates (monkey and guinea pig esophagus), the assessment of the exact antibody profile (anti-Dsg 1 and/or anti-Dsg 3) is important to establish the subtype of pemphigus. The frequency of pemphigus antibody occurrence in healthy relatives seems not to be incidental, but further studies should be performed to explain this phenomenon.

Adolescent↗

[Systemic sclerosis: symptomatic treatment].

Systemic sclerosis is an autoimmune connective tissue disease resulting in a fibrosis of skin and internal organs. The pathogenesis is unclear and includes genetic and environmental factors leading to an immune activation, vascular abnormalities, fibroblast proliferation and collagen deposition. The treatment of systemic sclerosis can be based on a model of its pathogenesis. Therapy includes vasoactive drugs, prevention of fibrosis or immunosuppression and symptomatic treatment. In our paper we present the proven remedies and novel therapies of systemic sclerosis--disease extremely difficult to treat because of changes in digestive, pulmonary, cardiovascular, neuromuscular and locomotor's systems.

Angiotensin-Converting Enzyme Inhibitors↗

[Systemic sclerosis: drugs and new therapeutic methods].

The purpose of our review is to present conventional drugs as well as innovative treatment concepts for systemic sclerosis, particularly with their effects on the most important pathogenetic mechanisms of this disease. Date from literature indicates that all therapeutic methods are connected with occurrence of side effects, therefore in our paper we focused on benefits and disadvantages of therapeutic options for systemic sclerosis.

Humans↗

Serum levels of tissue inhibitor of metalloproteinases 2 in systemic sclerosis: a preliminary study.

BACKGROUND: The etiopathogenesis of systemic sclerosis (SSc)--an autoimmunological disease characterized by excess collagen and other connective tissue components in skin and internal organs--remains unclear. Given the potential role of matrix enzymes, metalloproteinases, and their tissue inhibitors in pulmonary fibrosis, we assayed the serum concentration of tissue inhibitor of metalloproteinases 2 (TIMP-2) in patients with SSc and explored its possible correlation with the duration of Raynaud's phenomenon, the intensity of sclerosis of the skin, and pulmonary lesions. MATERIAL/METHODS: We studied 18 SSc patients, 8 with limited SSc and ten with diffuse. The degree of sclerosis of the skin was measured using the Total Skin Scale. Chest x-rays and spirometric examinations were also performed. The presence and type of antinuclear antibodies (ANA) were determined by indirect immunofluorescence and double immunodiffusion in agar gelatin. Serum TIMP-2 concentration was measured by ELISA. RESULTS: The average serum TIMP-2 concentration was not significantly higher in SSc patients than in controls. No statistically significant difference was found in this respect between limited and diffuse SSc. Despite the lack of correlation between the duration of Raynaud's phenomenon, the degree of sclerosis of the skin, and the serum concentration of TIMP-2, the latter concentration was higher in patients with restrictive pulmonary dysfunction in spirometric testing. CONCLUSIONS: Our research, though involving a small group of patients, points to the probable role of TIMP-2 in the development of pulmonary fibrosis.

Adolescent↗