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Results for “Scleroderma, Systemic”

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PubMed1980

[New developments in the pathology, pathophysiology and therapy of systemic scleroderma].

In scleroderma, collagen and elastic fibres as well as the dermo-epidermal junction are pathologic. The reactivity of the blood vessels, the function of the smooth muscle cells and the factors protecting against edema formation are defective. The urinary excretion of metabolites of collagen and ground substance glycosaminoglycans indicates quantitatively disease activity. After experimental mass testing in tissue culture, a group of inhibitors of the biosynthesis of essential connective-tissue components were transferred to therapy of scleroderma. In 89 percent of 115 patients, progression could be arrested. After several years of treatment, a regression came about in 74 percent of which 41 percent was subtotal and 17 percent total.

Amino Acids↗
PubMed1995

[Antiendothelial antibodies in systemic scleroderma and Raynaud's disease].

Using ELISA on fixed endothelial hybridoma (EAhy.926) cells we investigated the occurrence of antiendothelial antibodies (AEA) in the sera from patients with scleroderma systematica (SS) (n = 70) and Raynaud's disease (RD) (n = 19). The mean IgG and IgA-AEA levels were significantly higher in the SS patients than RD patients (p < 0.001) and controls (p < 0.001). We have detected circulating IgG-AEA in 64.2% of patients with SS, 35.2% of SS sera were positive for IgA-AEA. In patients with RD, the frequency of AEA corresponded to that of the random population sample. We have found a high incidence of Raynaud's phenomenon, myositis, telangiectasia and marked digital ischaemia (digital ischemic pulp ulcers, digital scars, osteolysis and autoamputation) among AEA positive patients with SS. AEA-positive patients were characterized by high extent and severity of Raynaud's phenomenon and higher average nailfold capillary microscopy scores. No correlations were found between AEA and different clinical or laboratory parameters, including the type of scleroderma (diffuse and limited), the presence of anti-Scl-7O and anticentromere antibodies and the clinical features of SS (lungs, kidneys and heart involvement, esophageal dysfunction, calcinosis, Sjogren's syndrome). There was no significant correlation between the AEA level and patient age, extent of skin involvement (skin score). Thus, in SS, AEA is associated with a peripheral vasculopathy and represent a useful marker for the diagnosis of endothelial dysfunction.

Adolescent↗
PubMed1995

[Myocardial scintigraphy with thallium-201 in the diagnosis of cardiac involvement in systemic scleroderma].

201Tl scintigraphy of the myocardium is a highly sensitive method of diagnosis of cardiac damage in SS. More severe lesions occur in patients with diffuse skin affections and subacute SS. Improvement of perfusion in dipiridamol test indicates the contribution of the spasm of microcirculation coronary vessels to the onset of perfusion defects. Further investigations are needed for evaluation of the role various vasodilators have in prevention of perfusion lesions and progressive myocardial fibrosis in SS patients.

Adult↗
PubMed1995

[The assessment of left ventricular diastolic function in patients with systemic scleroderma by radionuclide ventriculography].

To assess left ventricular (LV) diastolic function in scleroderma systematica (SS) with regard to SS course and skin lesion degree, a total of 24 SS patients were examined versus control subjects. Though cardiac symptoms manifested clinically only in 8 patients, parameters of LV diastolic function underwent changes in the majority of the examinees: the time to attaining maximal filling velocity increased, contribution of the first diastolic third to LV filling diminished, hemodynamic significance of the left atrial systole grew. More pronounced dysfunction of the myocardium occurred in patients with diffuse skin lesions, acute and subacute disease who also appeared to have reduced LV ejection fraction. The conclusion is made on frequent subclinical pattern of myocardial involvement in SS patients which runs primarily as LV diastolic dysfunction. Systolic disturbance emerge later or in more active forms.

Acute Disease↗
PubMed1994

[The clinico-functional characteristics of pulmonary arterial hypertension in systemic scleroderma].

Electro-, phono-, echo-, and Doppler echocardiography, bulbar biomicroscopy were performed in 54 SS patients aged 18-64. Mean pulmonary arterial pressure (PP) was determined at Doppler echocardiography on Toshiba SSH-40A unit according to techniques developed by M. M. Mirrakhimov et al. in 1989. The diagnosis of pulmonary hypertension (PH) was made in PP above 18 mm Hg. A close relationship was found between SS history and PH registration. With growing SS duration, PH severity increases, microcirculatory disorders progress. Main PH risk factors in SS patients are believed SS duration, activity, Raynaud's syndrome presentation. Doppler echocardiography can effectively diagnose PH in its subclinical stage, is noninvasive.

Adolescent↗
PubMed1995

[Gastrointestinal involvement in progressive systemic scleroderma].

The complained gastrointestinal symptoms in PSS are probably caused by several complex disturbances like intestinal transit disturbances (ITD), bacterial overgrowth of the small intestine caused malabsorption of bile acids and altered kinetics of intestinal hormones. 25 patients with PSS and eleven healthy controls were tested for the existence of ITD by use of the metal-detector test (MDT). Twelve patients were also tested for a malabsorption of primary bile acids by radioimmunological measurement of clolylglycine serum levels before and after a meal. In addition serum concentrations of gastrin (nine patients) and plasma concentrations of cholecystokinin (CCK) (eight patients) and motilin (eleven patients) were measured by radioimmunoassay pre- and postprandial. Interdigestive gastric emptying was accelerated in patients with PSS (53 +/- 3 min. vs. 73 +/- 7 min.; p<0.01). Small intestinal transit times were similar in both groups (115 +/- 17 min. vs. 121 +/- 13 min.). Colonic transit in patients with PSS was significant prolonged (63 +/- 6 h vs. 39 +/- 5 h; p<0.05). There were no significant differences between the two groups concerning the pre- and postprandial levels of cholylglycin. Basic and postprandial levels of gastrin, CCK and motilin were higher in the PSS group. In contrast to scintigraphic studies using semisolid meals gastric emptying of the copper pellet in PSS was accelerated. A general malabsorption of primary bile acids was not found. Prolonged colonic transit times correlate well with frequently complained obstipation. Gastric hypacidity could be the reason of elevated gastrin levels. The high motilin-levels in PSS could be due to a lack of the feed-back inhibition as a result of diminished phase-III activity of the interdigestive migrating motor complex. The elevation of CCK-levels could reflect compensation of neurogenic or myogenic disturbances of gallbladder contraction.

Adult↗
PubMedDate not supplied

[Haptoglobin and ceruloplasmin in systemic scleroderma].

Content of haptoglobin and ceruloplasmin was estimated in blood serum of 23 patients with sclerodermia systematica. A statistically distinct decrease in ceruloplasmin concentration was observed in blood of the patients, whereas content of haptoglobin was markedly increased; this phenomenon reflects a level of the pathological process activity. The proteins are synthesized in various zones of liver lobes: haptoglobin--in the central zone, ceruloplasmin - in the peripheral one. Decrease in content of ceruloplasmin in blood of patients with sclerodermia systematica was due to impairment of hepatocytes, localized in peripheral parts of liver lobes.

Animals↗