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Results for “Scleroderma, Systemic”

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PubMedDate not supplied

[Anti-nucleic acid antibodies in children with systemic lupus erythematosus and systemic scleroderma and in their relatives living with the probands].

The nature of antibodies to nucleic acids was studied in children of a Caucasian region suffering from systemic lupus erythematosus (SLE) and systemic sclerodermia (SSD). Two types of antibodies (to two- and one-filament DNA) were revealed in SLE, and in SSD mainly--to one-filament DNA. Antibodies to RNA in patients with SLE were specific for two-filament conformation. Immunogenesis disorders of different degree with respect to DNA and RNA were found; this expressed itself in the synthesis of various classes of immunoglobulins to these antibodies (IgG to DNA and IgM to RNA). High incidence of the RNA-binding activity of relatives living together with probands is noted. These data point to a high occurrence of antibodies to denatured DNA and low incidence of the cases of DNA-binding activity in the blood sera of the patients' fathers and brothers. Thus a combination of three factors: viral, genetic and hormonal in the pathogenesis of this disease is possible.

Adolescent↗
PubMed2004

[Level of soluble tumor necrosis factor receptor type I in patients with systemic scleroderma].

AIM: To study content and clinical correlations of sTNF-RI in patients with systemic sclerosis (SS). MATERIAL AND METHODS: Thirty nine SS patients were examined with enzyme immunoassay for serum levels of sTNF-RI and soluble adhesion molecules (sSAM) sVSAM-1, sISAM-1 and sR-selectine using R&D System kits (USA). The control group consisted of 14 healthy subjects (donors). Content of sTNF-RI and sSAM was considered as elevated if it exceeded relevant mean values by 1SD. RESULTS: sTNF-RI content was significantly higher in the patients than in the controls (p = 0.0001) and was similar in patients with diffuse and limited forms of the disease. A rise in sTNF-RI correlated with a rise in pulmonary artery pressure. In patients with pulmonary hypertension or restrictive pulmonary affection sTNF-RI was higher than in patients free of pulmonary hypertension or impaired external respiration function. A marked correlation was found between sTNF-RI and sVSAM-1. Patients with high CRP had significantly higher sTNF-RI level than patients with normal CRP. CONCLUSION: SS is characterized by elevated content of sTNF-RI. This content may serve a diagnostic marker of the disease progression.

Adult↗
PubMed1989

T lymphocyte subset abnormalities and HLA antigens in scleroderma (systemic sclerosis).

Studies of T lymphocyte subsets were carried out in a group of 50 scleroderma patients, of whom 46 were also HLA phenotyped. The total lymphocyte count was depressed in 22 patients, and CD4 (helper cells) numbers were normal. CD8 (suppressor-cytotoxic) cells were reduced in 27 patients, and the CD4/CD8 number ratio increased above normal in three additional patients, resulting in 30 patients being classified as CD8-deficient. In the 46 patients HLA phenotyped, DRw8 was significantly increased in the entire patient group, but when the patients were subdivided into CD8-deficient (n = 29) and CD8-normal (n = 17), the increase in DRw8 was confined to the CD8-deficient patients. B18 was also increased in patients with limited sclerosis, while DR4 and DRw53 were significantly decreased and DR5 significantly increased in patients with more extensive skin sclerosis. These findings suggest that scleroderma is a heterogeneous condition and that this heterogeneity is reflected in different HLA profiles in patients subtyped according to their clinical profile and subpopulations of T cells.

Adult↗
PubMed1991

[Esophagitis in progressive systemic scleroderma. Prevalence and risk factors in forty-six patients].

Forty-six patients with progressive systemic sclerosis (37 women and 9 men) were successively evaluated by endoscopy, manometry, and esophageal pH monitoring. Fourteen patients (30.4 percent) had erosive esophagitis. Twenty-four patients were symptomatic; nineteen patients complained of dysplagia. Erosive esophagitis was significantly more frequent in symptomatic patients than in asymptomatic patients (50.0 percent vs 9 percent, P less than 0.01) and especially in patients complaining of dysphagia (57.9 percent vs 11.1 percent, P less than 0.01). Erosive esophagitis was not correlated with symptoms of gastroesophageal reflux. Abnormal esophageal motility was found in 34 patients (73.9 percent). Occurrence of erosive esophagitis was not linked with esophageal dysmotility. In patients with erosive esophagitis lower esophageal sphincter pressures were significantly lower than those in patients without erosive esophagitis. Twenty-four hr-pH monitoring showed pathological gastroesophageal reflux in 20 patients (43.5 percent). Erosive esophagitis was more frequent in patients with pathological gastroesophageal reflux than in patients with normal gastroesophageal reflux (50.0 percent vs 15.4 percent, P less than 0.02) especially in patients with pathological supine nighttime gastroesophageal reflux (61.5 percent vs 18.2 percent, P less than 0.01). Our data suggest that symptoms, dysphagia, diminished lower esophageal sphincter pressures, and pathologic nighttime gastroesophageal reflux are reliable predictors of the presence of erosive esophagitis in patients with progressive systemic sclerosis.

Adult↗
PubMedDate not supplied

[Collagen metabolism in systemic scleroderma].

Rates of biosynthesis, maturation and degradation of collagen were studied in skin of patients with systemic sclerodermia. The rate of collagen biosynthesis was increased about 3-fold in most patients and in several persons--increased 4--6 fold. Collagen maturation was approximately twice increased in systemic sclerodermia. Content of collagen was decreased in skin of the patients and the rate of collagen degradation exceeded distinctly that of controls. The rate of collagen biosynthesis did not depend on the type of skin impairment. With development of sclerodermic disease maturation of collagen was increased and the rate of its degradation was, by contrast, decreased. The rate of collagen biosynthesis was the highest in chronic and progressive forms of sclerodermia. In subacute sclerodermia the rate of collagen biosynthesis was decreased as compared with chronic and progressive forms of the disease but it was higher than in normal state. Processes of biosynthesis and maturation of collagen were coupled, related to each other in subacute sclerodermia and in indurative skin. These processes were uncoupled in visually unimpaired skin under progressive and chronic sclerodermia.

Adolescent↗
PubMed1983

[Antinuclear, anti-DNA and anti-lymphocyte antibodies in systemic scleroderma. 62 cases].

The authors describe a prospective study of serum immunological abnormalities in 62 cases of systemic sclerodermia. Antinuclear antibodies were found in 67 percent of the cases. Contrary to expectation, the homogenous type was the most common. Anticentromeric antibodies were found in 4 of the 10 CREST syndrome patients. Anti-RNP (2/62) and natural anti-DNA antibodies are rarely found in sclerodermia. Non-cytotoxic anti-lymphocytic antibodies were found in more than half of the serums studied. They were frequently found in the CREST cases and in cases associated with Gougerot-Sjögren syndrome. Their significance is unknown. Lastly, almost all of the cases of systemic sclerodermia showed an immunological serum abnormality.

Antibodies, Antinuclear↗
PubMed1990

[Vasodilator agents in pulmonary artery hypertension of systemic scleroderma].

An acute haemodynamic study was carried out prospectively in 6 patients fulfilling the diagnostic criteria of systemic sclerosis with pulmonary arterial hypertension. This study consisted of 7 baseline measurements and the effect of 6 drugs administered intravenously and known for their effects as vasodilators in the literature. These results were compared to those obtained on 44 patients suffering from pulmonary arterial hypertension previously studied at Hospital A.-Béclère. The prostacyclin (PGI2) possesses a very potent vasodilator effect, lowering the total pulmonary vascular resistance by 29% in patients suffering from scleroderma. As well as PGI2 phentolamine and hydralazine seemed to possess an acute vasodilator action which is certainly not negligible.

Adult↗
PubMed1990

[Pulmonary manifestation of progressive systemic scleroderma: prognostic value of centromere antibodies and antibodies to Scl-70 nucleoprotein].

We examined 74 patients with systemic sclerosis. 21 of them (= 28%) had Scl-70 antibodies in their blood serum and 12 (= 16%) were anticentromere antibody positive, whereas in 41 cases (= 56%) none of these antibodies could be detected. In patients with Scl-70 antibodies lung function tests showed a decrease in vital capacity (p less than 0.05), total capacity (p less than 0.01), and diffusing capacity (p greater than 0.05/n.s.) as compared to ACA-positive patients. A limitation of pulmonary function was seen in 76% of Scl-70-positive patients, in 44% of antibody-negative patients, and in only 33% in the ACA-positive group. A disabling respiratory limitation was found in 14% of Scl-70-positive patients, and in 7% of antibody-negative patients, whereas none of the ACA-positive patients had a higher degree of respiratory insufficiency. We conclude that pulmonary involvement in systemic sclerosis is most frequent and severe in patients with Scl-70 antibodies; it is relatively rare and of minor severity in ACA-positive patients. Antibody-negative patients are in between these extremes.

Adolescent↗
PubMed1998

[The correlation between salivary endothelial expression of E-selectin and clinical and biological parameters in systemic scleroderma].

OBJECTIVES: A body of evidence suggests the pivotal role of endothelial cells in the pathophysiology of systemic sclerosis. E-selectin is an adhesion molecule specifically expressed by activated endothelial cells. In previous studies we noticed that E-selectin was frequently expressed in the salivary gland tissue of patients with systemic sclerosis. Moreover, E-selectin expression was detectable very early in the course of the disease. To better define the role of E-selectin in the pathogenesis of systemic sclerosis, we conducted a study aimed at determining whether E-selectin expression was correlated to clinical and biological features in patients with systemic sclerosis. METHODS: Thirty-one patients presenting with systemic sclerosis were included in the study. The following parameters were systematically assessed: duration and cutaneous extent of the disease, presence of secondary Sjögren's syndrome, antinuclear antibodies, and pulmonary and esophagus involvement. E-selectin expression was assessed by immunocytochemistry on minor labial salivary glands. RESULTS: E-selectin expression was detected in 21 out of 31 patients (67%). The disease duration was significantly shorter in patients with E-selectin expression (mean 9.1 +/- 8.5 years versus 4.2 +/- 3.3 years, P < 0.05). No significant difference was found for other features. CONCLUSIONS: This study shows that endothelial E-selectin expression is mainly detectable early in the course of systemic sclerosis, when active and non-cicatrical sclerosis may be evidenced. No correlation was found between E-selectin expression and immunological disorders (antinuclear antibodies, secondary Sjögren's syndrome).

Adolescent↗
PubMed1991

Circulating androgens in male patients suffering from systemic scleroderma.

The excretion of collagen metabolites and circulating androgens was measured in ten males suffering from progressive systemic sclerosis. Significantly higher levels of sex hormone-binding globulin, total testosterone and dihydrotestosterone (DHT) (P less than 0.01), and total oestradiol (P less than 0.05), were found in patients when compared with age-matched controls. Urinary excretion of hydroxyproline in patients was found to correlate significantly with total testosterone (P = 0.035), with DHT (P = 0.005) and dehydroepiandrosterone-sulphate (DHEAS) (P = 0.034). Similarly the hydroxyproline peptide fraction was found to correlate significantly with total testosterone (P = 0.037), with DHT (P = 0.005) and with DHEAS (P = 0.008). Hydroxylysine peptide in the urine correlated significantly with free testosterone (P = 0.035) and DHT (P = 0.040). Oestrogens did not correlate with urinary excretion of collagen metabolites. These findings suggest that androgens may play a role in the pathogenesis of scleroderma in male patients.

Adult↗
PubMed1994

Decreased bradykinin binding sites in fibroblasts from progressive systemic scleroderma.

The numbers of bradykinin receptors (BK-R) in cultured dermal fibroblasts from patients with progressive systemic sclerosis (PSS) and from healthy controls were measured using a receptor binding assay. The numbers of BK-R were significantly fewer in PSS fibroblasts than in control fibroblasts (P < 0.02). However, no differences in affinity were observed in BK-R between PSS and control fibroblasts. The BK-R mRNA levels were determined in PSS and control fibroblasts by Northern blot hybridization using BK-R cDNA, but no significant differences were found. These findings suggest that the decrease in BK-R in PSS fibroblasts might occur during a posttranslational step.

Binding Sites↗
PubMed1989

[Ungueal capillaroscopy in fasciitis with eosinophilia: a distinctive feature of systemic scleroderma. Apropos of 15 cases].

Eosinophilic fasciitis (EF) is a recently described disease whose distinction from progressive systemic sclerosis (PSS) is still being discussed. PSS has a characteristic microcirculation pattern. We performed nailfold microscopy on 15 patients with EF and compared the results to those of 98 PSS patients and 75 normal control subjects. EF patients have a normal microcirculation pattern (13/15) or discrete, non-specific anomalies: none had the typical capillary pattern associated with PSS and associated diseases. The findings of this study justify making a distinction between EF and PSS and demonstrate that nail fold microscopy can be a useful tool for an early differential diagnosis between these two disorders.

Adult↗
PubMed1985

Fibronectin distribution in nailfold biopsies of scleroderma (systemic sclerosis) patients.

The distribution of fibronectin (FN) was studied in skin biopsies of 13 patients with scleroderma (SD), 7 patients with dermatomyositis (D), and 10 normal controls (NC) by direct immunofluorescence. In normal tissues, continuous or segmental linear staining of the dermal-epidermal junction (DEJ) was seen. Papillary, subpapillary dermis, and papillary capillary loops showed a reticular pattern of deposition with fibronectin. Scleroderma patients revealed similar staining in the dermis and DEJ. The reticular distribution of FN appeared to stain more intensely in the dermis than in controls, especially in deeper layers. The amount of FN in walls of blood vessels from SD patients was markedly increased; all dermal vessels stained with FN and revealed considerably thicker walls and larger lumens. FN distribution in DM patients was similar to that seen in SD with an increased amount of FN staining in capillary walls.

Adolescent↗
PubMed1996

[Antibodies to collagen type I and fibronectin in patients with scleroderma systemic ].

Antibodies to collagen type I, total and immunoactive plasmic fibronectin were measured in 39 patients with scleroderma systematica using solid-phase enzyme immunoassay. It was found that growing activity of the disease was associated with an increase in the titer of antibodies to collagen type I and a decrease in the level of immunoactive fibronectin. With enhancement of the process generalization, the titer to the antibodies also rose. Total fibronectin in patients with scleroderma systematica and normal subjects was the same.

Adult↗