SEARCH · PubMed Health

Results for “Scleroderma, Systemic”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 73 records · Page 4Linked to original sources

PubMed1999

Fibroblast expression of collagen integrin receptors alpha1beta1 and alpha2beta1 is not changed in systemic scleroderma.

The skin of patients with systemic scleroderma (SSc) is characterized by excessive extracellular matrix deposition in the dermis. As collagens represent the major structural component, we used fluorescence-activated cell sorter analysis to study the levels of collagen receptors expressed at the surface of fibroblasts derived from involved skin areas. In contrast to previous reports, no differences in the expression of alpha1, alpha2 or beta1 integrin subunits, which constitute the major collagen receptors on fibroblasts, were detected on SSc fibroblasts as compared with normal control fibroblasts. Variation of cell culture conditions, e. g. passage number (from 2 to 10), seeding density, cell cycle or serum concentration, did not change this result. These observations indicate that any abnormal response of SSc fibroblasts to their matrix environment is not controlled at the level of receptor expression.

Adult↗

PubMed1975

[Research of an association between HL-A antigens and systemic scleroderma].

49 unrelated subjects suffering from systemic scleroderma were typed for 28 HL-A antigens, without any particular significant association of an antigen with the disease or one of its manifestations being noted. In addition, 13 patients from the same family were genotyped for HL-A. Transmission of the disease through 4 generations does not seem to be linked to a particular haplotype and no pair of sibling HL-A identical patients were seen in the same generation. By contrast, two pairs of sibling patients were HL-A different. Nevertheless, other cases, and in particular familial, will be necessary before an association between the genes of susceptibility to S.S. and a gene in the chromosomal HL-A region may be definitely eliminated.

Adult↗

PubMed1991

[Changes in the lungs in progressive systemic scleroderma].

In 24 patients with progressive systemic scleroderma the respiratory disturbances, X-ray, scintigraphic and clinical deviations as well as the statistical correlations between them are described. The functional examination of respiration revealed lowered diffusion capacity, restrictive type of ventilatory failure and hypoxemia as the most frequent disturbances. The functional examination of respiration is correlated with the X-ray changes in the lungs in these patients.

Acid-Base Equilibrium↗

PubMed1985

[Corinfar in the treatment of Raynaud's syndrome in systemic scleroderma (preliminary results)].

Twenty patients with systemic scleroderma (SSD) and Raynaud's syndrome (RS) received 30-80 mg of corinfar, a blocker of calcium channels, during 14 days. Seventeen patients responded to the treatment. The clinical effect was manifested by a decrease in the rate, duration and intensity of RS attacks. Side effects were recorded in 6 patients, but the drug was discontinued only in 2 patients because of the allergic reactions. During treatment, the patients showed an increase in tissue blood flow parameters. Corinfar may be regarded a drug of choice in the treatment of marked RS in SSD.

Adult↗

PubMed1987

Silica-dust-exposed mine workers with scleroderma (systemic sclerosis).

The incidence of scleroderma (systemic sclerosis) was found to be increased in a population of black men who were gold miners. Ten men with scleroderma were detected during a five-year period. The annual incidence of the disease in this population in the group aged 33 to 57 years was estimated to be 81.8 per million. All of the men with scleroderma had disturbances of pulmonary function which were not present in a control group of silica-dust-exposed men without scleroderma. Not all of the subjects with scleroderma had silicosis, but all had been occupationally exposed to silica dust. There was a significant increase in the prevalence of tuberculosis in the past in the group with scleroderma, compared with a group of men with silicosis from the same population. The nature of the association of tuberculosis with scleroderma has not been defined.

Adult↗

PubMed1990

[Mild infrared A hyperthermia in treatment of systemic scleroderma].

Seven female systemic sclerosis patients (all from acrosclerosis type, with intestinal involvement, and marked Raynaud phenomenon) were treated with infrared A whole body irradiations (wavelengths between 800 and 1,400 nm, 12 W/dm2 maximally). The single exposure lasted for 30 minutes and resulted in an 0.9 degrees C rise of central body temperature. Acral skin rewarming became regular immediately after irradiation and kept improved, as compared with pre-treatment values, for at least 18 weeks. All the patients told about a comfortable feeling of warmth after each treatment lasting for one two days. Three out of the seven reported lower frequency and severity of Raynaud attacks.

Female↗

PubMed2005

Systemic scleroderma: a spatiotemporal clustering.

BACKGROUND AND AIMS: The aetiology of systemic scleroderma remains poorly understood. Twin studies suggest a low genetic input. Of the incriminated environmental agents, silica and vinyl chloride monomer exposure appear the most convincing. Spatiotemporal clustering has been demonstrated only three times previously. We now report a fourth cluster around Edenhope, western Victoria in terms of numerator and denominator estimates, cumulative incidence, distribution in time and space, and possible aetiological factors. METHODS: Prevalence/cumulative incidence numerator and denominator values were obtained and validated. Each case was age-and gender-matched with two controls. A standardized postal questionnaire was used to obtain data on current, past history, family history, and occupational and non-occupational environmental exposure. RESULTS: Six systemic scleroderma cases and one mixed connective tissue disease patient with a predominance of scleroderma features were identified. The 5-year cumulative incidence was 6.1/10,000, tenfold higher than the Sydney estimates for a similar, though non-identical time period. The gender ratio was 1:1. No cases were genetically related. A family history of scleroderma was validated in one instance and a family history of Raynaud's was noted in first degree relatives of two cases and one control. In all instances, Edenhope residence preceded disease onset. No one environmental agent was implicated in all cases. CONCLUSION: A spatiotemporal cluster of systemic scleroderma was confirmed and validated. It occurred with a tenfold increased cumulative incidence to that expected and also extended beyond the initially defined 50 km radius of Edenhope. The cases identified were not related. Although no one specific environmental agent was identified, the spatiotemporal clustering would be compatible with an agent occurring at relatively high frequency, but with low disease conversion rates, such as silica inhalation (assuming sufficiently small particle size) or reaction to an infective agent.

Aged↗

PubMed2002

[Pathogenesis of systemic scleroderma: immunological aspects].

Systemic sclerosis (SSc) is a connective tissue disorder that is characterized by excessive collagen synthesis by fibroblasts and by vascular hyperreactivity and obliteration phenomena. Excessive collagen production is the consequence of abnormal interactions between endothelial cells, fibroblasts and mononuclear cells. Immunological abnormalities are present very early in the development of SSc. Mononuclear cells, particularily macrophages and T lymphocytes play a prominent role in fibroblast activation and collagen synthesis through the cytokines they produce. Thus, lymphocytic infiltrates in the skin and in the lung are preferentially composed of CD8+ T lymphocytes, that produce important amounts of interleukin 4 (IL-4). The effects of IL-4 are added to these of transforming growth factor B (TGF-B) and connective tissue growth factor (CTGF) that stimulate collagen synthesis by fibroblasts. T lymphocytes produce important amounts of gamma interferon (INF-gamma) that is the best inhibitor of collagen synthesis by fibroblasts. However, the inhibitory effect of INF-gamma on collagen synthesis is diminished in SSc patients. Numerous autoantibodies can be evidenced in the serum of SSc patients. Three of them are specific for SSc and mutually exclusive: anti-centromere antibodies (Ab) in limited SSc, anti-Scl70 Ab in diffuse SSc and anti-RNA polymerase III Ab in diffuse SSc with renal involvement. These autoantibodies are good prognosis markers but their pathogenic role remains uncertain.

Autoantibodies↗

PubMed1978

Scleroderma-inducing glycosaminoglycan in the urine of patients with systemic scleroderma.

A glycosaminoglycan with scleroderma-inducing effect was isolated and partially purified from the urine of patients with systemic scleroderma. The glycosaminoglycan was an N-sulfated glycosaminoglycuronan and its high total sulfate and 2,5-anhydromannose contents suggest that the glycosaminoglycan is a degradation product of heparin or polysulfated heparin sulfate. Furthermore, the composition of the above glycosaminoglycan was similar to that of the N-sulfated glycosaminoglycan which we observed previously in uninvolved skin of scleroderma.

Adult↗

PubMed2001

Chronic constipation--a lethal danger in patients with systemic scleroderma.

The gastrointestinal tract is one of the main sites of manifestation of systemic scleroderma. We describe a case of a 62-year-old patient with systemic scleroderma and progressive severe constipation that led to stercoral obstruction and perforation. We make a brief review of the literature on the gastrointestinal manifestations and the management of systemic scleroderma.

Chronic Disease↗

PubMedDate not supplied

[Current approaches to the treatment of patients with systemic scleroderma].

Principles are highlighted of modern therapy of systemic scleroderma involving prescription of basis agents, such as, in the first place, thiol compounds as well as drugs affecting certain links of the disease pathogenesis, in particular, antifibrotic, vasodilative, anti-inflammatory ones. Methods are presented of therapy of systemic scleroderma with unithiol and superelectrophoresis of unithiol with dimexide, developed by the author. Syndrome therapy of the disease is characterized taking account of damage to certain organs and systems.

Adrenal Cortex Hormones↗

PubMed1997

[MRI of fingers in systemic scleroderma. Initial results with contrast-enhanced studies using a dedicated MRI system].

PURPOSE: To estimate disease activity in patients with systemic sclerosis using contrast-enhanced MRI of the skin. MATERIAL AND METHODS: In a pre-study, sequences of a low-field (0.2 T) scanner (Artoscan, Esaote, Genova, Italy) were optimized for detection of intravenous contrast (0.1 mmol/l Gd-DTPA) in six patients with the autoimmune disease systemic scleroderma. Based on the results of the pre-study, 17 patients with scleroderma (7 sclerotic/10 active inflammatory disease) were scanned using gradient-spoiled 3D GRE sequences (FA 90 degrees, TR 100 ms, TE 18 ms), which had been established as most sensitive for intravenous contrast. Contrast enhancement of the skin was determined quantitatively by contrast-to-noise ratios (CNR), comparing post- to pre-contrast and dynamic scans (for 6 min, 1 acquisition/min). Patients in the chronic state with sclerodactylia and active inflammation of the hands were considered separately and compared to a control group (n = 10) matched according to age. RESULTS: CNR increase after intravenous contrast was significantly higher in patients with active disease (86 +/- 16% increase) than sclerosing disease (29 +/- 3%, p < 0.05) and the control group (4 +/- 2%, p < 0.05). The dynamic examination showed a significantly slower decrease after the peak rise in the first minute in patients with active disease (CNR 15.4 +/- 0.7 to 14.2 +/- 1.4) than in those with chronic disease (14.1 +/- 0.5 to 11.3 +/- 0.9, p < 0.05). DISCUSSION: Capillary leakage is the most likely explanation for the increased enhancement in patients with active scleroderma. Using sequences optimized for contrast detection, disease activity in the course of scleroderma and response to therapy can be determined by MRI in the future.

Adult↗

PubMed1996

Pigmentation abnormalities in systemic scleroderma examined by using a colorimeter (Choromo Meter CR-200).

Cutaneous colors of the dorsum of the hands (A), the distal forearms (B; 5 cm from the wrists), the proximal forearm (C; proximal 1/3 from the elbow) and sternal skin region (D) in patients with systemic scleroderma (73 cases; M:F = 16:57) systemic lupus erythematosus (SLE) or dermatomyositis (27 cases; M:F = 7:20) and healthy controls (HC) (36 cases; M:F = 8:28) was characterized by a XYZ colorimetric system (CIE, 1931) using a colorimeter (Choromo Meter CR-200, Minolta Camera Co. Ltd., Osaka). The index Y, which means color value shows a lower value in male HC and in patients with systemic scleroderma, especially in the more severe type with hyperpigmentation (score 5-6; the system proposed by Ishikawa) than that of female HC. The values of indices x and y, which relate to reddish (erythema with hyperpigmentation) and greenish color (pale), respectively, were higher in the exposed portion of the severe type of systemic scleroderma with hyperpigmentation, especially male and older patients, and in unexposed portions of the female group without hyperpigmentation. Histopathologically, there was prominent pigmentation in the upper dermis of the forearm in the severe type of systemic scleroderma, so that melanin quantity may be closely related to the decrease in index Y. There was no statistical significance in the value of indices Y, x and y between HC, SLE and dermatomyositis. This method may contribute not only to diagnosis of systemic scleroderma and differentiation from other collagen diseases, but also studies of clinical follow-up and effects of medication.

Adult↗

PubMed1986

Type III collagen aminopropeptide levels in serum of patients with progressive systemic scleroderma.

Sera from 101 patients with progressive systemic scleroderma were analyzed for circulating aminopropeptides of type III collagen using a radioimmunoassay which measures the intact and degraded forms (Fab assay). About 41% of the patients were found to have values above the normal range. A good correlation was observed between elevated levels of aminopropeptides and the degree of involvement of the skin and internal organs in the patients. Most patients (89%) with an active progression of the disease but not those in a stationary phase showed increased serum levels of aminopropeptides. Treatment with corticosteroids apparently normalized the levels of aminopropeptides. Only minor changes were observed with an antibody-based radioimmunoassay which measures primarily the intact form of the aminopropeptide.

Adult↗

PubMed2003

[Vascular pathology in systemic scleroderma].

The mechanisms of vascular lesions in systemic scleroderma (SSC) are still little studied with the current comprehensive investigations being focused on this issue. The results of a study dealing with the structural-and-morphological and molecular reasons of sclerodermic micro-angiopathy as compared with the variations and pattern of the clinical disease course are summarized in the article. The structural capillary changes were evaluated on the basis of the results of a wide-field video-capillaroscopy of the nail bed (CNB). The level of soluble adhesion molecules VCAN-1, ICAM-1 and P-selectine determined by the quantitative immune-enzyme assay described the vascular endothelium condition. Morphological examinations of dermal samplings included an identification of the lymphocytic composition of infiltrates by applying the mononuclear antibodies to markers T (CD3, CD4, CD8) and B (CD20) of lymphocytes and detection of the endothelial activation by applying the mononuclear antibodies to intercellular adhesion-1 molecule (ICAM-1). The conducted investigations revealed the structural capillary changes in all SSC patients; the nature of such changes is closely related with a clinical variation and course of the disease. The morphological signs of micro-angiopathy were detected in 98% of patients including at the early disease stage. A more pronounced perivascular infiltration with predominance of CD4+ T-lymphocytes was observed and expression of ICAM-1 to the endothelial cells was registered more often in an active disease course. Higher levels of VCAM-1, ICAM-1 and P-selective in blood were found in 80%, 45% and 48% of patients, respectively. Correlations of VCAM-1 with an activity and a progressing disease course were established. Therefore, the serological and morphological signs of vascular lesions reflect an intensity degree of sclerodermic micro-angiopathy and correlate with an SSC clinical course.

Adolescent↗

PubMed1991

[Eosinophilia as a possible heart damaging factor in systemic scleroderma in children].

Altogether 173 children with systemic scleroderma (SSD) were examined. Of these, 45 were with eosinophilia. It was discovered that associated SSD and eosinophilia ran a course marked by more well-defined exudative reactions, with the heart being injured more frequently and gravely. A correlation was noted between the "sclerodermic" heart and the eosinophil count in the peripheral blood. The relationship between eosinophilia and the content of the total serum IgE in SSD is discussed as is the role of age-associated and constitutional factors in the development of eosinophilia in SSD children.

Age Factors↗

PubMedDate not supplied

[Value of echocardiography in the diagnosis of cardiac lesions in systemic scleroderma].

A total of 20 patients with systemic scleroderma (SSD) were examined with the use of a single-measure and sectoral echocardiography. Despite the absence of clinical signs of circulatory insufficiency a disorder of intracardiac hemodynamics with the indices of the central hemodynamics being normal were revealed in the examined patients. Latent exudative pericarditis producing no substantial effect on the hemodynamics was diagnosed in four patients. Fibrosis of the valvular apparatus was observed in three patients. It has been found advisable to include echocardiography into complex examination of patients with SSD.

Adult↗

PubMed1988

[Systemic scleroderma in children].

A new case of systemic scleroderma in a child is reported. The frequency of this disease is much lower than focal scleroderma and than other connective diseases in childhood. Kidney and heart involvement is much less common than in adults. Raynaud's phenomenon and scleroderma however, as in this case, are often severe. The appropriate treatment has to be efficient towards sclerosis (D-penicillamine, corticosteroids), Raynaud's phenomenon (protection from cold, nifedipine) and other manifestations (prevention of gastric ulceration, physical therapy) and should consider every possible adverse effect on a growing organism.

Adrenal Cortex Hormones↗