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

Trudności terapeutyczne u pacjenta z pęcherzycą liściastą

A case of 49-year old male, who manifested extensive, varied in size, erythematous skin lesions with shallow erosions, on trunk. A diagnosis of pemphigus foliaceus was stated upon clinical manifestations, direct immunofluorescence examination, indirect immunofluorescence examination and enzyme-linked immunosorbant assay for desmoglein 1 antibodies in serum. The patient was initially treated with prednisone and azathioprine, according to consensus of Polish Dermatological Society. After two weeks of treatment, the patient was hospitalized in surgery ward, due to escalated stomach-ache. In the ward, esophageal mycosis and inflammation of the mucuous membrane of the stomach were observed and considered as treatment’s complications. Due to adverse events observed, second line therapy was administered: cyclophosphamide and methylprednisolone (initially in pulses, than orally). Improvement in clinical manifestations was noted.Przedstawiono przypadek 49-letniego pacjenta z rozległymi, różnej wielkości zmianami skórnymi o charakterze ognisk rumieniowych z powierzchownymi nadżerkami, zlokalizowanymi głównie na tułowiu. Na podstawie obrazu klinicznego oraz badań immunofluorescencyjnych (bezpośredniego i pośredniego oraz immunoenzymatycznego) rozpoznano u chorego pęcherzycę liściastą. Początkowo zalecono leczenie zgodne z obowiązującym konsensusem Polskiego Towarzystwa Dermatologicznego (prednizon i azatiopryna). Z powodu nasilonego bólu brzucha po dwóch tygodniach leczenia pacjent był hospitalizowany na oddziale chirurgii, gdzie rozpoznano powikłania leczenia w postaci grzybicy przełyku i zapalenia błony śluzowej żołądka. Ze względu na obserwowane objawy niepożądane zmodyfikowano leczenie ogólne. Po zastosowaniu leczeniu drugiego rzutu (cyklofosfamid oraz metyloprednizolon, początkowo w pulsach, następnie doustnie) nastąpiła poprawa

Opryszczkowate zapalenie skóry o nietypowym obrazie klinicznym

A case of 33-year old female with dermatitis herpetiformis, who manifested vast, erythema-edematous skin lesions with peripheral vesicles feston and rosette -shaped like. Skin lesions developed after dapson was discontinued in therapy. A diagnosis of dermatitis herpetiformis was verified, as clinical manifestations were suggesting a diagnosis of linear IgA bullous dermatosis. Direct immunofluorescence examination revealed granular IgA deposits (+++) in the dermal papillae. This result supported a diagnosis of dermatitis herpetiformis and excluded a diagnosis of linear IgA bullous dermatosis.    Przedstawiono przypadek 33-letniej pacjentki chorującej na opryszczkowate zapalenie skóry, u której po wyłączeniu z leczenia dapsonu wystąpił masywny wysiew zmian rumieniowo-obrzękowych i pęcherzyków o obrączkowatym i festonowatym (rozetowatym) układzie. Ze względu na obraz kliniczny sugerujący rozpoznanie linijnej IgA dermatozy pęcherzowej zdecydowano o weryfikacji diagnozy. W badaniu metodą immunofluorescencji bezpośredniej stwierdzono ziarniste złogi IgA (+++) ułożone w szczytach brodawek skórnych, co wykluczyło rozpoznanie linijnej IgA dermatozy pęcherzowej, potwierdziło zaś diagnozę opryszczkowatego zapalenia skóry

Expression of Selected Integrins and Selectins in Bullous Pemphigoid

Blister development in bullous pemphigoid (BP) results from destruction of hemidesmosomes and basement membrane components within the dermoepidermal junction by autoantibodies. Adhesion molecules can take part in pathogenesis of this disease. The aim of the study was to determine the localization and expression of L- and E-selectins and β1, β3, and β4 integrins by immunohistochemistry in skin lesions of 21 patients with BP, compared with 10 healthy subjects. Expression of L and E selectins and β1, β3 integrins was detected mainly in basal keratinocytes and in inflammatory infiltrates in the dermis, expression of β4 integrin was irregular and was detected mainly in dermal part of the blister, while in the control group only weak and single expression of the examined molecules was detected in basal keratinocytes and endothelium cells. The obtained results reveal the important role of selected selectins and integrins in development of skin lesions in BP

The artificial oxygen carrier erythrocruorin—characteristics and potential significance in medicine

The diminishing supply and increasing costs of donated blood have motivated research into novel hemoglobin-based oxygen carriers (HBOCs) that can serve as red blood cell (RBC) substitutes. HBOCs are versatile agents that can be used in the treatment of hemorrhagic shock. However, many of the RBC substitutes that are based on mammalian hemoglobins have presented key limitations such as instability and toxicity. In contrast, erythrocruorins (Ecs) are other types of HBOCs that may not suffer these disadvantages. Ecs are giant metalloproteins found in annelids, crustaceans, and some other invertebrates. Thus far, the Ecs of Lumbricus terrestris (LtEc) and Arenicola marina (AmEc) are the most thoroughly studied. Based on data from preclinical transfusion studies, it was found that these compounds not only efficiently transport oxygen and have anti-inflammatory properties, but also can be modified to further increase their effectiveness. This literature review focuses on the structure, properties, and application of Ecs, as well as their advantages over other HBOCs. Development of methods for both the stabilization and purification of erythrocruorin could confer to enhanced access to artificial blood resources

Rola limfocytów T regulatorowych w patogenezie wybranych chorób pęcherzowych

Regulatory T cells (Treg) are a phenotypically heterogeneous group ofcells, playing an important role in regulation of the immune response.To date, several subtypes of regulatory cells have been identified. Themost important of them are: lymphocytes CD4+CD25+, Tr1, Th3 andNK cells. Regulatory T lymphocytes act by secretion of suppressivecytokines (IL-10, TGF-β) or direct cell-to-cell interaction. Functionalabnormalities of regulatory T cells are important in pathogenesis ofmany diseases, especially autoimmune ones. Based on published studies,the review discusses the role of regulatory T cells in pathogenesisof selected bullous skin diseases

The Role of Angiogenesis Factors in the Formation of Vascular Changes in Scleroderma by Assessment of the Concentrations of VEGF and sVEGFR2 in Blood Serum and Tear Fluid

Systemic sclerosis (SSc) is a connective tissue disorder characterized by tissue hypoxia, excessive fibrosis of skin and internal organs, and angiogenesis imbalance. The aim of the study was to evaluate in SSc patients the association between the retinal microcirculation disturbances and the presence of peripheral trophic changes and to determine the role of angiogenesis factors in the formation of vascular changes in scleroderma. Twenty-five SSc patients and 25 age- and sex-matched healthy controls were included to the study. Assay of vascular endothelial growth factor (VEGF) and soluble VEGF receptor-2 (sVEGFR-2) in blood serum and tears was done for all patients and controls using enzyme-linked immunosorbent assay. Retinal blood circulation was investigated with fluorescein angiography (FA) in the SSc patients only. In our research, proportion of mainly hypertensive patients presenting with a large spectrum of retinal microvascular lesions was 72%, while proportion of patients with skin microvascular lesions within distal phalanxes of fingers and toes was 76%. We noticed that patients with pathological changes in the FA examination had finger ulcerations significantly more often than patients without changes in the eye fundus. There were no statistically significant differences in the serum concentration of VEGF and sVEGFR2 between subjects in both analyzed groups. Analysis of lower levels of VEGF (p=<0.001) and sVEGFR-2 (p=<0.001) in blood serum accompanied by simultaneous higher levels of VEGF/sVEGFR-2 ratio in tears of SSc patients, as compared with the control group, indicates the superiority of proangiogenic factors in patients’ tears