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PubMedDate not supplied

Nutritional support in patients with systemic scleroderma.

Systemic scleroderma often involves the gastrointestinal tract and can lead to significant malnutrition. Specialized home nutritional support was provided to five patients (one enteral and four parenteral) with restoration of adequate nutritional status, improved quality of life, and few metabolic or technical complications over a period of 12 to 86 months. Three patients eventually died of their disease, one was converted to an oral diet, and one patient remains dependent on total parenteral nutrition. This experience would recommend consideration of home nutritional support in patients with systemic scleroderma when the disease is relatively stable and no major organ failure is present.

Adult↗
PubMed2001

Juvenile systemic scleroderma.

Systemic scleroderma in children is very rare and is considered similar to adult-onset disease. In adults, new etiopathogenetic and therapeutic approaches have emerged in recent years. For instance, it has been shown that microchimerism could play a role in disease pathogenesis and that immunoablation followed by stem cell rescue could be of potential therapeutic benefit. There is also evidence that these new approaches can be of value for childhood-onset disease.

Adolescent↗
PubMed1999

Investigation of sialic acids and sialyltransferase activity in blood of patients with systemic scleroderma.

Systemic scleroderma (SSd) is a connective tissue disorder accompanied by generalized fibrosis. A disturbance of the synthesis and production of matrix glycoproteins, such as collagens, fibronectin, and proteoglycans, by connective tissue cells is typical for this disease. We previously demonstrated a decrease in the ganglioside content of cultured skin fibroblasts from patients with SSd. In this work the contents of sialoglycoproteins and sialoglycolipids in blood sera of patients with SSd were estimated. Simultaneously, the level of asialofetuin-sialyltransferase activity in blood plasma of three groups of patients--those with SSd, Raynaud's phenomenon, and with localized scleroderma--was investigated. CMP-5-acetamido-9-deoxy-9-fluoresceinylthioureidoneuraminic acid was used as a substrate for the enzyme assay. It was shown that the concentration of total sialic acid was increased and the concentration of lipid-bound sialic acid was slightly decreased in the blood sera of patients with SSd. A correlation between the lipid-bound sialic acid level and the severity of disease was observed; there was no correlation between severity of disease and total sialic acid. Sialyltransferase assay showed a decrease in the activity level in all three groups of patients. The greatest decrease (2-fold) of this activity was observed in patients with Raynaud's phenomenon. Our data suggest that in SSd and similar diseases the process of glycoconjugate sialylation is disturbed. These changes may considerably affect the mechanisms of regulation of metabolism and cellular interactions.

Cells, Cultured↗
PubMed1992

[Heart involvement in systemic scleroderma].

Systemic scleroderma is a general disease of the connective tissue which may affect practically any organ. The authors describe the case of a man where in the course of the disease affection of the heart with symptoms of severe cardiac failure became the dominating symptom.

Aged↗
PubMed1992

[Relation between 67-gallium scintigraphy and bronchoalveolar lavage--differential cell count and superoxide anion liberation--in patients with systemic scleroderma and systemic lupus erythematosus].

The aim of the study was to determine the pulmonary 67-gallium uptake, bronchoalveolar lavage (BAL) cell differentiation and the activity of BAL cells, measured as release of superoxide anion (O2-), and to investigate the results whether there are relations. In 11 nonsmoking systemic scleroderma (SS) patients and 11 systemic lupus erythematosus (SLE) patients with lung involvement double-sided BAL were performed, mainly in regions with increased 67-gallium uptake. Release of O2- was measured by INT-assey. BAL cell differentiation was in SS and SLE pathological in 68.2% without side-difference. In contrast to SS, O2(-)-release in SLE depends on BAL cell differentiation and is most increased in normal BAL cell differentiation. There is no correlation between 67-gallium uptake and both BAL cell differentiation and O2(-)-release. The results suggest, that in contrast to SS, BAL cells in SLE with pathological differentiation are less activated than BAL cells with normal differentiation probable due to autoimmunological factors. Pulmonary 67-gallium scan and BAL seem to be independent from each other.

Adult↗
PubMed2000

[Strategy for early diagnosis of lung involvement in systemic scleroderma].

Systemic sclerosis (SSc) is an autoimmune disorder characterized by accumulation of collagen in affected organs, mainly the skin and the lungs, associated with abnormalities of the arterioles and capillaries. There are two types of pulmonary involvement, which influence long term prognosis: infiltration of the lungs and/or pulmonary artery hypertension. Full investigations into possible lung involvement must be performed systematically when SSc is diagnosed and during follow-up. The double pathophysiology sometimes makes diagnosis difficult but it must be made as early as possible in order to decide on the optimal treatment. The aim of this study was to evaluate the usual explorations and to propose biological markers to identify patients requiring more detailed lung investigations, in order to establish a diagnostic approach to treatment and follow-up patients with SSc.

Aftercare↗
PubMed1991

[Bronchoalveolar lavage in systemic scleroderma and systemic lupus erythematosus--differential cell values and enzyme cytochemistry].

The aim of the study was to determine the bronchoalveolar lavage (BAL) cell differentiation and the activity of beta-glucuronidase and N-acetyl-beta-D-glucosaminidase in alveolar macrophages. In 12 patients with systemic sclerosis (SS), 4 with systemic lupus erythematosus and 4 healthy controls BAL was performed. The activity of beta-glucuronidase and N-acetyl-beta-D-glucosaminidase was measured semiquantitative by means of cytochemical methods. Lymphocytes and neutrophils in BAL cell differentiation are increased, also the activity of beta-glucuronidase. The activity of N-acetyl-beta-D-glucosaminidase is decreased in SS and SLE in comparison with controls. The activity of beta-glucuronidase seems to be a marker of activity of alveolar macrophages in SS and SLE.

Acetylglucosaminidase↗
PubMed2001

[Echocardiographic functional assessment of the heart and lipid metabolism in patients with systemic scleroderma and systemic lupus erythematosus].

AIM: To characterize structural-functional state of the heart by means of uni-, two-dimentional and doppler echocardiography in impulse regimen; to evaluate lipid metabolism in patients with scleroderma systematica (SS) and systemic lupus erythematosus (SLE). MATERIAL AND METHODS: The examination of 35 SS and 38 SLE patients included clinical examination, echocardioscopy with doppler cardiography, measurements of serum concentrations of total cholesterol, triglycerides, low and high density lipoproteins, atherogenic index. RESULTS: In SS patients central hemodynamics reflects hyperdynamics. In intact contractility, diastolic function of the ventricles is impaired. Elevated atherogenicity of the serum was found in both the groups. CONCLUSION: According to echocardiography SLE and SS are associated with changes in both intracardiac and central hemodynamics. High atherogenicity of the serum may appear an additional factor of risk of hemodynamic disturbances.

Adult↗
PubMed1991

[Bronchoalveolar lavage in patients with systemic scleroderma and systemic lupus erythematosus: characterization of cell activity by cytofluorometry, chemiluminescence and differential cell count].

The purpose of the study was to determine the role of lymphocyte subsets (Ly) and reactive oxygen metabolites RSM, concerning the activity of BAL cells, in the pathogenesis of lung involvement in 12 patients with systemic sclerosis (SS) and 4 with systemic lupus erythematodes (SLE) in comparison with 10 control subjects. The cellular activity was measured by means of cytofluorometry (CFM) and chemiluminescence (CL). In SS/SLEY CD3+, CD4+, CD4/CD8-ratio, CD25 + T-Ly and luminol-dependent CL are increased (p less than 0.05). Correlations exist between CD3+, CD4+, CD8+ and CD25 + T-Ly and both luminol-dependent CL and neutrophils (p less than 0.01). The results suggest, that increased secretion of RSM by BAL-cells may be caused by local release of lymphokines by these activated T-Ly. Therefore CFM and CL seem to be useful in addition to BAL cell differentiation in characterizing the BAL cell activity in the diagnostic of lung involvement in SS and SLE.

Adult↗
PubMed2001

[Multivariate analysis as a method of determination of anthropogenic load on the course of Raynaud's disease in patients with systemic scleroderma and systemic lupus erythematosus].

AIM: To determine the effect of high industrial pollution on development of Raynaud's syndrome and impairment of pulmonary hemodynamics in patients with scleroderma systematica (SS) and systemic lupus erythematosus (SLE). Two groups of patients were examined: 20 SS and 33 SLE patients living in highly polluted territories, 15 SS and 27 SLE patients living in moderately contaminated territories. The tests included capillaroscopy, thermography, rheovasography of the fingers, zonal rheopulmonography. Semiquantitative spectral analysis measured content of 3 trace elements (Ti, Ba, Li) and 16 heavy metals (Cu, Zn, Pb, Ni, Mn, Sn, Cr, Y, Ga, Ag, Mo, Bi, Co, Cd, Fe, Zr) in the patients' hair. RESULTS: Patients living in highly polluted areas had a severe course of Raynaud's syndrome. Metal accumulation is decisive in patients of group 1; in development of staging of Raynaud's syndrome the key role belongs to lead, nickel, tin and iron; realization of the factor of the disease activity is related to lowering of zinc, copper concentrations and growing concentration of nickel. CONCLUSION: Vascular disorders in SS and SLE in patients living in heavily contaminated areas aggravate due to direct damage from heavy metals.

Environmental Pollutants↗
PubMed1989

Isolated pulmonary hypertension in the grandchild of a kindred with scleroderma (systemic sclerosis): "neonatal scleroderma"?

We report a small kindred in which the father and daughter with positive antinuclear antibodies (ANA) (proband) had diffuse cutaneous and visceral scleroderma (systemic sclerosis, SSc), antitopoisomerase autoantibodies and shared the HLA-A23 C- B- DR5 DRw52 DQw3 haplotype. The ANA- granddaughter (daughter of the proband) was noted to have severe isolated pulmonary hypertension within the first 6 months of life, and had the other maternal HLA-A1 Cw8 B14 DR1 DQw1 haplotype, which included a B1, B2 duplication of the C4B allele. All 3 members shared DRw52. The possibility that neonatal pulmonary hypertension represents an isolated autoimmune disease or a hitherto undescribed neonatal syndrome is proposed and the immunogenetic autoantibody implications are discussed.

Antibodies, Antinuclear↗
PubMed2000

Diffuse and limited cutaneous systemic scleroderma.

Juvenile systemic scleroderma (jSSc) is a rare disease. Based on the first large data collection on this patient group, the disease course was demonstrated to differ from that in adults. The concept of persistence of maternal cells in patients with SSc remains pathogenetically fascinating, as does the resemblance with graft-versus-host-disease. In view of new therapeutic options, controlled trials have not established a gold standard for treatment, but autologous bone marrow transplantation may be considered a rescue therapy for selected patients. Palliative therapies have also improved markedly in recent years. The first controlled trials for patients with jSSc are being proposed.

Adult↗
PubMed1995

HLA and autoimmunity in scleroderma (systemic sclerosis).

Scleroderma or systemic sclerosis (SSc) has been associated with certain class II antigens of the major histocompatibility complex (MHC), including HLA-DR1, DR2, DR3, DR5, and DR52. In general, these earlier HLA correlations were weak and varied considerably among reporting centers and different ethnic populations. More recently, a variety of disease-specific autoantibodies have been discovered including anti-centromere, antitopoisomerase I, and a variety of anti-nucleolar antibodies. These specificities show little overlap among one another, and each are markers for certain clinical features of SSc. At the same time, molecular studies of the MHC have provided more accurate methods for defining specific HLA alleles. Now it is becoming clear that certain HLA class II alleles, especially HLA-DQ, are more strongly associated with autoantibody subsets of SSc than with the disease itself. For example, anticentromere antibodies are strongly associated with HLA-DQB1*0501 (DQ5), DQB1*0301 (DQ7) and other DQB1 alleles possessing a glycine or tyrosine residue in position 26 of the outermost domain. Anti-topoisomerase I antibodies occur in SSc patients with HLA-DQB1*0301 (DQ7), DQB1*0302 (DQ8), DQB1*0601 (DQ6 in Japanese), and other DQB1 alleles possessing a tyrosine residue in position 30. HLA-DQ alleles associated with these autoantibodies tend to be in linkage disequilibrium with the HLA-DR specificities previously associated weakly with SSc itself. Rare multiplex families with SSc also show these same HLA haplotypes co-segregating with autoantibody profiles in affected members. Thus, it appears that MHC alleles play a role in affecting the serological expression of SSc, and the implications of these recent findings are discussed.

Alleles↗
PubMed1997

[Cardiovascular manifestations in systemic scleroderma].

OBJECTIVES: Patients with systemic scleroderma often have latent heart disease which could play an important role in morbidity and mortality. We therefore conducted a prospective study of cardiovascular manifestations in patients with systemic scleroderma. PATIENTS AND METHODS: A prospective cross-sectional study included 29 patients with systemic scleroderma who underwent a complete cardiovascular work-up including physical examination, electrocardiogram, chest x-ray and Doppler-echocardiogram from July 1993 to February 1996. RESULTS: Hypertension was observed in 6 patients (20.7%) and was positively correlated with age (p = 0.007). Raynaud syndrome was also found in 6 patients (20.6%). Heart disease was observed in 14 patients (48.3%) and was positively correlated with age and lack of treatment for scleroderma (p = 0.008). Myocardial disease was the most frequent (11 patients, 37.9%), followed by pericardial disease and valve disease (4 cases each, 13.8%). Rhythm and conduction disorders were found in 2 (6.9%) and 8 (27.6%) of the patients. CONCLUSION: Cardiovascular manifestations are frequent but often latent in patients with systemic scleroderma. This finding emphasizes the importance of routine cardiovascular work-up in all patients with scleroderma.

Adolescent↗