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Results for “Scleroderma, Systemic”

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PubMed1989

[Anti-Scl-70 and anti-centromere autoantibodies. Biological markers of 2 forms of systemic scleroderma].

The diagnostic and prognostic value of anti-Scl-70 autoantibodies in systemic scleroderma and other connective tissue diseases was investigated. A clinical and immunological study consisting of a search for anti-Scl-70 autoantibodies by immunodiffusion and immunoblot and a search for anti-centromere autoantibodies and antinuclear factors by indirect immunofluorescence was conducted in 57 cases of systemic scleroderma, 45 cases of CREST syndrome, 41 patients with suspected systemic scleroderma but who did not respond to the American rheumatism association (ARA) criteria, 35 cases of systemic lupus erythematosus, 8 cases of polymyositis, 40 cases of Raynaud's phenomenon and 48 controls. Anti-Scl-70 autoantibodies were found by immunodiffusion in 40 and by immunoblot in 45 cases of scleroderma with lesions proximal to the metacarpo-phalangeal joint (a major ARA criterion) and in 3 patients with suspected scleroderma but only one minor criterion: sclerodactyly. In systemic scleroderma with proximal lesions, the anti-Scl-70 autoantibody has a sensitivity of 0.85 and a specificity of 0.99. Anti-centromere autoantibodies were present in 39 cases of complete or incomplete CREST syndrome, 3 cases of Raynaud's phenomenon probably evolving towards a connective tissue disease and 1 case of systemic scleroderma with proximal lesions. The anti-Scl-70 and anti-centromere autoantibodies seem unable to coexist in the same patient and appear to be markers of two forms of scleroderma with different courses and prognoses. The Scl-70 antigen, which is the target of the anti-Scl-70 antibody, has been identified as topoisomerase 1, and a functional abnormality of this enzyme might contribute to some of the chromosomal abnormalities described in scleroderma.

Autoantibodies↗

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↗

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↗

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↗

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↗

PubMed1991

Clinical aspects of localized and systemic scleroderma.

A number of reports of potential etiologic agents of localized and systemic scleroderma appeared in the past year, including alterations in tryptophan metabolism, use of appetite suppressants, and exposure to silicone. An infectious agent, Borrelia burgdorferi, was found not to be implicated in localized scleroderma. The improvement in outcome of systemic scleroderma complicated by renovascular hypertension was highlighted in several papers, as was the emerging importance of cardiac and pulmonary involvement. Recent advances in the early detection and evaluation of cardiac and pulmonary complications of scleroderma are discussed.

Humans↗

PubMed1978

Antibodies against extractable nuclear antigens (ENA) in systemic scleroderma.

Antibodies against nuclear ribonucleoprotein (RNP) were found in 5 of 63 cases of systemic scleroderma, whereas they were present in all but one case of mixed connective tissue disease and in 15 of 67 cases of systemic lupus erythematosus. In all RNP positive cases of systemic scleroderma there were some features of other collagen diseases, and their course was relatively more benign. Studies of RNP antibodies in systemic scleroderma may be of importance for treatment and prognosis.

Antibodies, Antinuclear↗

PubMed1990

[A comprehensive study of heart function in patients with systemic scleroderma].

The data of instrumental studies in 43 patients with systemic scleroderma were compared to the clinical picture, which made it possible to specify the character and to reveal new regularities of heart lesions in patients with the above disease. The instrumental research methods, echo- and polycardiography in particular, allow an objective control of heart lesions in systemic scleroderma which should be specified in making the diagnosis and in the course of the follow-up of patients.

Adolescent↗

PubMed1989

[Microcirculation in patients with systemic scleroderma during treatment using hyperbaric oxygenation].

Hyperbaric oxygenation treatment of systemic scleroderma has a favourable effect on microcirculatory changes whose positive dynamics can be demonstrated by conjunctival biomicroscopy. These changes include accelerated blood flow and decrease in the degree of erythrocyte aggregation. The method can be used for the objective assessment and for prognosis of the effectiveness of hyperbaric oxygenation treatment in patients with systemic scleroderma.

Adult↗

PubMed1990

Antikinetochore and antitopoisomerase I antibodies in systemic scleroderma: comparative study using immunoblotted recombinant antigens, immunofluorescence, and double immunodiffusion.

In 135 patients with systemic scleroderma, we compared three different methods to determine antinuclear autoantibody (ANA) specificity: indirect immunofluorescence, double immunodiffusion, and, employing recombinant antigens, immunoblotting using both marker autoantigens of this disease. A characteristic Scl-70 antibody pattern was found on HEp-2 cells in 83.8% of the patients, double immunodiffusion was positive for the Scl-70 antibodies in 81.9%, and immunoblot with the recombinant topoisomerase I (Topo I) was positive in 71% of the patients. For the centromere autoantibodies we found a high concordance between the anticentromere antibody (ACA) pattern on HEp-2 cells (27 patients positive) and the detection of recombinant kinetochore in immunoblotting (26 patients positive). The three testing techniques gave comparable results, except that the Topo I recombinant antigen used in immunoblotting reacted strongly with fewer than expected of the known Scl-70-positive sera. However, a method using recombinant antigens expressing all epitopes (rather than one of the epitopes of Topo I) will undoubtedly become the method of choice for detecting antibodies in systemic scleroderma. Using the immunoblotting technique with the recombinant antigens we detected in four patients antibodies against both Topo I and kinetochore. More severe symptoms of systemic scleroderma were found in patients who had both antibodies. The combined presence of both marker autoantibodies is therefore not as rare as previously reported and may predict severe disease.

Adolescent↗

PubMed1977

Hepatocyte giant mitochondria: an almost constant lesion in systemic scleroderma.

Liver electron microscopic studies were performed in 14 patients with systemic scleroderma. In 13 of these patients, giant mitochondria were demonstrated in the hepatocytes. This ultrastructal abnormality was present whatever the type and duration of the disease and was also present even when the liver was histologically normal. The mechanism of formation of giant mitochondria in systemic scleroderma is unknown.

Adult↗

PubMed1991

Immunologic markers of systemic scleroderma in children.

This study was performed on seven children with systemic scleroderma, three with the diffuse and four with the limited type. All three patients with diffuse scleroderma had high titers of clumpy pattern antinucleolar antibody on HEp-2 cells. The course of the disease was severe, and two children died. Four children with limited scleroderma had mild disease, and Scl-70 antibody, an immunologic marker that in adults is associated mostly with diffuse scleroderma. In one child Scl-70 antibody and anticentromere antibody coexisted, although previously the two were believed to be mutually exclusive. This study shows that limited scleroderma of childhood with slight cutaneous involvement may be associated with the Scl-70 marker. The findings in 10 adults in whom Raynaud's phenomenon developed in childhood and indurations appeared some years later, point to the significance of careful observation of these children, with repeated testing for immunologic markers of SSc. An important new finding is the association of different types of systemic sclerodermas with specific immunologic markers.

Adolescent↗

PubMed1986

Enhanced angiogenic capability of monocyte-enriched mononuclear cell suspensions from patients with systemic scleroderma.

Different subsets of peripheral blood mononuclear cells (MNC) from 15 patients with systemic scleroderma were tested for their ability to evoke angiogenesis in a xenogenic system. The angiogenic capability of total MNC from patients with systemic scleroderma was lower than that of normal human cells, irrespective of the form of the disease. However, the capability of a monocyte-enriched subset of MNC from patients with scleroderma was found to be increased, as compared with their total MNC and with that of the corresponding subset from healthy individuals. This might be due to the activation of monocytes in the disease.

Adult↗

PubMed1979

Lymphocyte responsiveness to urinary glycosaminoglycan in systemic scleroderma.

From the urine of patients with systemic scleroderma, we previously isolated a glycosaminoglycan, which could induce a scleroderma-like change in the skin of mouse. In the present work, the cell-mediated response to urinary glycosaminoglycans was examined by lymphocyte transformation test. The specific response was observed in lymphocytes of patients with scleroderma, when the above scleroderma-inducing glycosaminoglycan was added. By contrast, lymphocytes of patients with SLE or dermatomyositis and of normal persons hardly showed a response to this glycosaminoglycan. On the other hand, the glycosaminoglycans from normal urine could not stimulate lymphocytes of patients with scleroderma or healthy persons.

Antigens↗