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Results for “Scleroderma, Systemic”

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PubMed1987

[Classification of progressive systemic scleroderma].

A new classification of forms of progressive systemic scleroderma (PSS) is presented. Compared with previous classifications, it includes not only frequent, typical forms of PSS, but also rarer manifestations. For the first time, it considers pathogenetic factors, such as the phenomena which have become known concerning the immunological system, and distinguishes between noninflammatory and inflammatory subtypes. Etiological (in this case, immunogenetic) criteria are also considered. This classification is open to further differentiation and development.

Antibodies, Antinuclear↗

PubMed1987

[The state of cellular immunity in patients with systemic scleroderma].

A study of 50 patients with autoimmune diseases (of them 35 suffered from systemic scleroderma, 10 from systemic lupus erythematosus, 4 from fibrosing alveolitis and 1 from Goodpasture's syndrome) showed the importance of the functional state of the immune system for pathogenesis of the disease. The predominance of DTH reactions expressed in leukocyte migration inhibition, was noted in patients with minimum activity of the process. The effect of serum factors on the patients' lymphocyte functional activity was shown.

Cell Migration Inhibition↗

PubMed2003

Effects of a nonsurgical exercise program on the decreased mouth opening in patients with systemic scleroderma.

The decreased mouth opening (microstomia) represents a frequent finding in patients with systemic scleroderma (SSD), but little information is available about the efficacy of nonsurgical management of this condition. The aim of this study is to assess the effects of a nonsurgical exercise program on the decreased mouth opening in a group of 10 SSD patients with severe microstomia (maximal mouth opening < or =30 mm). The subjects were instructed to perform an exercise program including both mouth-stretching and oral augmentation exercises. The effects of such exercises were assessed after an 18-week period by measuring the maximal mouth opening of each subject. All patients completed the study and no adverse effects occurred, with the exception of transient muscular fatigue. The exercise program improved the mouth opening of all subjects (mean increase: 10.7+/-2.06 mm, P<0.005), without significant differences between dentate and edentulous ones (P>0.1). At the end of the 18-week period, all patients commented that eating, speaking and oral hygiene measures were easier. The edentulous subjects also experienced less difficulty inserting their own dentures. These findings suggest that regular application of the proposed exercise program may be useful in the management of microstomia in SSD patients.

Adult↗

PubMed1983

Activation of fibroblasts induced by mononuclear cells from patients with systemic scleroderma and inactivation by mitogen stimulation.

Unstimulated mononuclear cell supernatants from patients with systemic scleroderma significantly augmented the DNA and collagen biosynthesis of 3T3 cells compared to normal mononuclear cells. This increase was suppressed by either a particular glycosaminoglycan, which from previous studies, has appeared to act as one of the tissue antigens in sclerotic skin, or concanavalin A stimulation.

Adult↗

PubMed1987

[Various aspects of the differential diagnosis of a pulmonary syndrome in systemic scleroderma and chronic aspecific lung diseases].

A comparative clinicoinstrumental study of the cardiorespiratory system in 29 patients with systemic scleroderma (SSD) and 56 patients with chronic nonspecific pulmonary diseases (CNPD) made it possible to specify the genesis of roentgenomorphological and functional pulmonary changes in SSD and to work out some clinical and functional tests to differentiate between the sclerodermic lung and CNPD. It was emphasized that differential diagnosis of the pulmonary syndrome in SSD and concomitant pulmonary pathology should be based on a combined study not only of clinical and x-ray findings but also of indices of pulmonary ventilation, hemodynamics of the greater and lesser circulation. A conclusion was made of the leading role of bronchial obstruction in the pathogenesis of hemodynamic pulmonary disorders in CNPD and the primary vascular and cardiac components in SSD.

Adult↗

PubMed1994

[Observation on blood flow changes in 34 cases of progressive systemic scleroderma treated with Chinese herbal medicine].

The blood flow change of 34 progressive systemic scleroderma (PSS) patients were examined. The amplitude proved to be lowered markedly than healthy subjects. All patients were treated with the basic prescription of PSS as the principal method, combined with infusion of Mailuoning injection in 500 ml of 5% glucose. The course of treatment lasted three months to one year. The result of treatment showed that the abnormal blood flow of extremities of all patients were improved remarkably. Marked improvement rate and total effective rate were 70.5% and 100% respectively. Significant improvements in clinical and laboratory parameters were observed. It revealed that there was a close relationship between the occurrence and development of PSS and blood circulation. It is assumed that the pathogenic mechanism of PSS is Deficiency of vitality and Excess of pathogenic factor (Stasis of Blood), and the Qi tonifying and Blood activating, hard lump softening and mass dissolving medicinal herbs according to Syndrome Differentiation of TCM should be used.

Adult↗

PubMed1988

Paget's disease of the breast with underlying carcinoma arising in systemic scleroderma.

A case is presented of Paget's disease of the breast with underlying infiltrating carcinoma arising in a 35-year-old woman with systemic scleroderma. The tumor arose in an area of the skin affected by the systemic scleroderma 4 years after the onset of her systemic disease. The possibility of a causal relationship between these two processes is discussed and a brief review of the literature is presented.

Adult↗

PubMed1991

[Nuclear medicine kidney diagnosis in progressive systemic scleroderma].

Kidney involvement is one of the most frequent causes of death in progressive systemic scleroderma (PSS). It is therefore important to detect potential impairment of renal function in PSS very early. In 76 patients referred for nuclear medicine diagnostic procedures, 42 pathologic results were found with 131I-hippurate clearance, while only 14 abnormal results were detected by static 99mTc-DMSA scans. Hippurate clearance is thus a sensitive method of functional renal evaluation in PSS.

Adolescent↗

PubMedDate not supplied

[Function of external respiration in patients with systemic scleroderma (according to roentgenopneumopolygraphic data)].

External respiratory function was investigated in 33 patients with systemic scleroderma+ using roentgenopneumopolygraphy (RPPG). Respiratory changes consisted in diffuse and regional ventilation disturbance (after emphysematous, hypoventilation or mixed type), disorders of respiratory kinetics of the diaphragm, ribs and mediastinum. External respiratory functional disturbance showed direct correlation with the patients' age, period of disease and a degree of pathological activity. The type of external respiratory functional disturbance was unchanged during observation over time. The use of exercise testing showed the presence of signs of latent ventilation insufficiency in most of the patients.

Adult↗

PubMed1986

[Individual aspects of pathogenesis and diagnosis of cardiorespiratory disorders in rheumatoid arthritis and systemic scleroderma].

The authors presented the results of a combined instrumental study of the cardiorespiratory system in 100 patients with rheumatoid arthritis (RA) and 26 patients with systemic scleroderma (SSD). Disturbances of the pulmonary circulation and central hemodynamics in the absence of roentgenomorphological and ventilation disorders in the bronchopulmonary apparatus were revealed. The dependence of hemocirculatory parameters on a degree of the expression and genesis of pulmonary changes was shown. Some reasons for a rare development of the pulmonary heart in rheumatic diseases were described. In the detection of bronchoobstruction in RA and SSD patients the use of drugs controlling it was recommended.

Adolescent↗

PubMed1987

Association of progressive systemic scleroderma to several HLA-B and HLA-DR alleles.

The HLA-A, B, C, and DR loci of 136 patients with progressive systemic scleroderma have been determined. The patients were classified according to the extent of their skin affection and into groups with or without immunologic and inflammatory signs of the disease. The antigens of the A locus did not show any significant deviations in frequency of occurrence. An increase of HLA-B8 and HLA-DR3 was only proved in the male patient group. Furthermore, in the HLA-DR gene locus, an increase in frequency of HLA-DR1, 2, 3, and 5 could be found. However, in the total set of patients, only the correlation of HLA-DR5 with progressive systemic scleroderma reached significance. Patients suffering from the CREST (calcinosis, Raynaud's phenomenon, esophagus, sclerodactyly, and telangiectasia) syndrome showed an increase of HLA-DR1. Patients with inflammatory signs of the scleroderma showed an accumulation of HLA-DR2. Several HLA-linked genes control the susceptibility to scleroderma.

Adult↗

PubMed1984

Circulating immune complexes in systemic scleroderma.

Circulating immune complexes were detected by the immunoelectrophoretic method in 18 of 29 (62 per cent) of patients with systemic scleroderma. The presence of immune complexes did not correlate with that of antinuclear antibodies to dsDNA, DNP, RNP, and Sm. The mean levels of immunoglobulins G, A, and M as well as of C3 were significantly higher in patients with systemic scleroderma than in blood donors.

Antibodies, Anti-Idiotypic↗

PubMed2001

Localized and systemic scleroderma.

Scleroderma is a broad term encompassing both localized and systemic sclerosis. Localized scleroderma is a cutaneous limited fibrosis that manifests as plaque morphea, generalized morphea, linear scleroderma, and deep morphea. Systemic scleroderma (sclerosis) can manifest as either limited or diffuse disease. Limited systemic sclerosis is typically preceded by Raynaud's phenomenon, involves cutaneous sclerosis distal to the elbows, with gastrointestinal and pulmonary fibrosis, and anticentromere antibody positivity. Diffuse systemic scleroderma is characterized by simultaneous Raynaud's phenomenon, cutaneous skin involvement proximal to the elbow with gastrointestinal, pulmonary, renal and cardiac fibrosis, and positive serology for antitopoisomerase and anti-RNAP III antibodies. This article discusses the classification, epidemiology, pathogenesis, clinical manifestations, treatment, and prognosis of the scleroderma.

Adult↗

PubMed1987

[Bronchiolo-alveolar carcinoma complicating systemic scleroderma under long-term treatment with factor XIII].

A 52-year old woman with systemic scleroderma had been treated since 1978 with factor XIII. The skin lesions had improved, and respiratory lesions had become stabilized. In 1984, she developed a bronchiolo-alveolar carcinoma which was treated by surgery. The authors emphasize that factor XIII is well tolerated and has beneficial effects on the skin and probably also on the lungs, without tachyphylaxis; however, the possibility of a bronchioloalveolar carcinoma occurring cannot be ruled out.

Adenocarcinoma, Bronchiolo-Alveolar↗

PubMed2005

[The diastolic dysfunction of the left ventricle in patients with systemic lupus erythematosus and system scleroderma].

The subjects of the study were 22 patients with systemic lupus erythematosus (SLE) and 18 patients with system scleroderma (SS). The mean age of the subjects was 36.3 +/- 2.4 years, the onset of the disease had taken place 5 to 10 years ago. The control group consisted of 20 practically healthy individuals with no complaints, clinical signs or instrumental data suggesting cardiovascular pathology. In order to evaluate the character of left ventricular (LV) diastolic filling, all the patients underwent transthoracal Doppler analysis with measurement of transmitral flow in four-chamber heart position using apical approach with the control volume at the level of the ends of mitral valvular cusps (computed sonography system ACUSON 128 XP/10). The study found no significant difference between SLE and SS patients in such parameters as LV myocardial mass and LV mass index. All the patients with rheumatic diseases, with or without arterial hypertension (AH), had diastolic dysfunction, which was manifested by increase of atrial systolic contribution into LV filling, prolongation of blood flow slowdown time in the stage of its early filling, and prolongation of LV isometric relaxation time; heart diastolic disorder was accompanied by significant increase of end diastolic pressure in LV cavity. It should be noted that the most prominent changes were found in rheumatic patients with AH, which must be caused by the hypertrophy and remodeling of the myocardium. Myocardial hypertrophy was associated with substantial changes in the ventricular septum, which consisted in its hypokinesia, associated with impairment of myocardial contractility (ejection fraction of 48.3 +/- 3.5%).

Adult↗

PubMed1992

[The clinical significance of detecting the inhibition of topoisomerase I by the sera of patients with systemic scleroderma].

Topoisomerase I activity was studied by electrophoresis in agarose gel according to plasmid DNA relaxation. Sera from 62 patients with systemic scleroderma, 35 with Raynaud's syndrome, 8 with focal scleroderma, 15 with systemic lupus erythematosus, 20 with rheumatoid arthritis and 20 healthy subjects were examined. Out of 62 sera from SSD patients, anti-topoisomerase activity was found in 67.8% of cases. The test appeared positive in 79% of patients with diffuse and 63% with limited disease patterns. The mean age and disease standing were similar in the positive and negative groups. An increase of the skin count and more frequent occurrence of trophic disorders in patients with inhibition of the enzyme were recorded. 40% of the patients demonstrated the coincidence of the results with the use of the topoisomerase test and ELISA. In patients with other rheumatic diseases and in the healthy subjects, no inhibition of the enzyme was found.

Animals↗

PubMed1987

Significant increase of urinary low-sulfated heparan-sulfate-related protein in patients with severe systemic scleroderma.

Radioimmunoassay with an antibody produced against urinary low-sulfated heparan-sulfate-related protein was devised and used to screen the heparan sulfate level in the urine of patients with systemic scleroderma. Patients with diffuse scleroderma, and patients also showing polymyositis/dermatomyositis had elevated values, whereas the value in patients with acrosclerotic scleroderma did not differ from that of the control population. In addition, an increase in this protein was associated with the positivity of anti-Scl-70 antibody. These findings suggest an important role for low-sulfated heparan sulfate in the pathobiology of severe systemic scleroderma.

Adult↗

PubMed1985

[Centromere antibodies and antibodies against Scl 70 nucleoprotein in progressive systemic scleroderma. Diagnostic and prognostic significance].

Frequency and diagnostic significance of two scleroderma-specific antinuclear antibodies were examined in 104 patients with progressive systemic scleroderma (PSS). Antibodies against an antigen in the centromere region of chromosomes (centromere antibodies) were demonstrated by indirect immuno-fluorescence on HEp-2 cells in 18 patients (17%) and antibodies against Scl-70 nucleoprotein (Scl-70 antibodies) by gel precipitation with soluble nuclear extracts (ENA) in 21 patients (20%). In none of the patients did both antibodies occur. In patients with Scl-70 antibodies severe illness was predominant with extensive cutaneous sclerosis in 86%, lung involvement in 85% and joint involvement in 89%. Humoral inflammatory signs were marked in this group. Patients with centromere antibodies predominantly had acroscleroderma (56%) with subcutaneous calcinosis (83%) and telangiectasias (83%), as known from the CREST syndrome. Humoral immune phenomena were rare. With regard to Raynaud's syndrome, involvement of the oesophagus, age and the duration of disease, there was no difference between the two groups. On the other hand, in patients with centromere antibodies lung involvement (22%) and joint involvement (35%) was significantly less common (P less than 0.01) than in all other scleroderma patients. Centromere antibodies and Scl-70 antibodies, which can be demonstrated already in the symptom-poor early phase of PSS, can thus be used as diagnostic parameters for different forms of PSS with differences in the degree of skin and organ involvement.

Adolescent↗