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Results for “Scleroderma, Systemic”

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PubMed1978

[Calcium ion uptake by the skin fibroblast culture under normal conditions and in systemic diseases of the connective tissue].

Radioactive calcium (45Ca) uptake and the effect of adrenaline on this process was studied on the suspension of cells derived from the monolayer cultures of fibroblasts of the skin of healthy donors and of patients with systemic scleroderma and rheumatic fever. A significant increase of calcium uptake was shown in case of scleroderma in comparison with the normal and rheumatism cases. Adrenaline (2 X 10(-7)--2 X 10(-5) M) enhanced the rate of calcium uptake by fibroblasts in control and had the opposite effect in scleroderma. The data obtained indicated alteration of the fibroblast membrane function in systemic scleroderma which could underlay the change in the metabolic activity of these cells.

Adolescent↗
PubMed1998

Management of severe scleroderma with long-term extracorporeal photopheresis.

BACKGROUND: The management of systemic sclerosis remains unsatisfactory. Thus far, the action of extracorporeal photopheresis (ECP) in severe systemic scleroderma has been evaluated in short-term studies, and only limited experience has been obtained with long-term application. OBJECTIVE: The aim of the present study was to evaluate prospectively the long-term effect of ECP in a group of 16 patients suffering from severe scleroderma, showing visceral involvement and progressive clinical course. METHODS: Fourteen patients with systemic scleroderma involving several organs, 1 with CREST syndrome and another with scleroderma-myositis overlap syndrome were treated with ECP over a period of 6-45 months. In 3 cases, gamma-IFN was additionally administered. Skin and visceral involvement were assessed by evaluating a series of clinical criteria and results from laboratory, imaging and functional tests. RESULTS: Overall, clear improvement was encountered in 6 patients, mixed response in 2, stable disease in 3 and continuing progressive course in 5 patients. Four out of 6 patients with improvement were treated with ECP early after onset of scleroderma (< or = 2 years), whereas all patients with a progressive course under ECP had had scleroderma for longer than 2 years. Immunosuppressive drugs previously administered could be reduced or fully withdrawn under ECP treatment in 5 patients, but additional oral medication was introduced in 4 patients due to disease progression. Addition of gamma-IFN to ECP did not reveal further benefit. No side-effects were recorded under ECP treatment. CONCLUSIONS: Based on this observation, we believe that long-term ECP represents an effective treatment modality in severe scleroderma particularly when started early, with stabilization of the disease course and partial remission of the cutaneous findings, whereas visceral involvement, if present, may rarely improve.

Female↗
PubMed2001

Systemic sclerosis (scleroderma). A case of recovery of cardiomyopathy after vitamin E treatment.

A 60-year-old woman with systemic sclerosis, systemic hypertension, and chronic renal failure, presented with clinical manifestations of heart failure. An echocardiogram showed a mildly dilated left ventricle and global hypokinesis. A six-month treatment including reduced sodium intake, furosemide, and nifedipine did not change the clinical and instrumental findings. Casually, vitamin E (600 mg daily) was added. After 6 months, clinical manifestations of heart failure were disappeared and the echocardiogram showed a normally-sized left ventricle with normal wall motion.

Antioxidants↗
PubMed1996

Anti-topoisomerase-I and clinical findings in systemic sclerosis (scleroderma).

The relationship between anti-topoisomerase-I antibodies and clinical findings was studied in 191 patients with definite systemic sclerosis. This was done by performing ELISA to detect antibodies to recombinant topoisomerase-I. Antibodies to topoisomerase-I were found in 72 patients (37%) with systemic sclerosis, which is a higher percentage than reported in most previous reports on a large unselected population. In 43 patients the presence of antibodies to recombinant topoisomerase-I was confirmed using both the immunodiffusion method and ELISA, with similar results. When classified into diffuse versus limited disease, a significant difference in antibody prevalence was demonstrated (P < 0.005), thus indicating that anti-topoisomerase-I antibody detection with ELISA may assist in early identification of systemic sclerosis subtypes.

Autoantibodies↗
PubMed1986

Prospective evaluation of thyroid function in patients with systemic sclerosis (scleroderma).

A consecutive series of 77 ambulatory, clinically euthyroid patients with systemic sclerosis was evaluated for clinical, chemical and serologic evidence of thyroid disease. Measurements of baseline serum thyroid function tests and the thyroid stimulating hormone response to thyrotropin releasing hormone (TRH) were made. We classified 18 (23%) patients as abnormal, including 8 (10%) who were chemically hypothyroid and 10 (13%) with normal baseline studies but an exaggerated response to TRH injection. The remaining 59 (77%) patients were euthyroid by all measurements. Antithyroid antibodies were present in only 4 of 8 (50%) of the hypothyroid group. There were no differences between the 3 patient groups with regard to the extent of scleroderma, the presence of internal organ involvement, or the frequency of common signs or symptoms of hypothyroidism. Abnormal thyroid function in systemic sclerosis is frequent, often unsuspected clinically, and may occur without markers of autoimmune thyroid disease.

Adult↗
PubMedDate not supplied

Phospholipids and glycosphingolipids in cultured skin fibroblasts from patients with progressive systemic sclerosis (scleroderma).

OBJECTIVE: A comparative investigation of glycosphingolipids and phospholipids of cultured skin fibroblasts from healthy donors and patients with progressive systemic sclerosis (SSc) was performed. METHODS: Culture techniques, qualitative and quantitative lipid analyses, determination of neuraminidase activity, and immunofluorescence microscopy were used. RESULTS: The quantitative content of individual phospholipids in the fibroblasts of SSc patients (PF) was equally elevated, on the average 2.8 times in comparison with the fibroblasts of healthy donors (DF). The total content of neutral glycosphingolipids was slightly elevated only because of a 1.5-fold increase of monoglucosylceramide in PF. A decrease in the amount of the gangliosides GM3 and GM1 and the absence of the disialogangliosides in PF in comparison with DF, were demonstrated. Immunofluorescent assay also showed a decrease of ganglioside epitopes on the cellular surface of PF in comparison with DF. The GM3-neuraminidase activity of PF homogenates was increased two-fold in comparison to normal values. CONCLUSIONS: These results are discussed in connection with abnormalities in membrane receptor functions, alterations in the cell phenotype and AMP-cyclase system activity, as well as hyperproduction of extracellular matrix proteins by PF.

Adult↗
PubMed2001

Nerve growth factor and neuropeptides circulating levels in systemic sclerosis (scleroderma).

OBJECTIVE: To determine the circulating levels of nerve growth factor (NGF), neuropeptide Y (NPY), and vasoactive intestinal peptide (VIP) in systemic sclerosis (SSc), and to correlate these levels with clinical and laboratory features. METHODS: Forty four patients with SSc were evaluated for circulating NGF (immunoenzymatic assay), NPY and VIP (radioimmunoassay), anticentromere and antitopoisomerase I autoantibodies, lung disease (pulmonary function tests with carbon monoxide transfer factor (TLCO), ventilation scintiscan with 99mTc DTPA radioaerosol, high resolution computed tomography (HRCT), pulmonary pressure (echo colour Doppler)), heart disease (standard and 24 ECG, echocardiography), cutaneous involvement (skin score), joint involvement (evidence of tender or swollen joints, or both), peripheral nervous system (PNS) involvement (electromyography), rheumatoid factor, angiotensin converting enzyme (fluorimetric method), von Willebrand factor (ELISA), and erythrocyte sedimentation rate (ESR) (Westergren). RESULTS: Circulating NGF levels in SSc were significantly increased compared with controls (p<0.00001) and significantly higher in the diffuse than in the limited subset of patients (p<0.01). Patients with articular disease had significantly higher levels of NGF. A significant indirect correlation between NGF levels and TLCO was detected (p<0.01), but no correlation was found between NGF and HRCT, DTPA, skin score, PNS involvement and angiotensin converting enzyme and von Willebrand factor levels, antitopoisomerase or anticentromere antibodies, and ESR. NGF levels increased progressively as the disease worsened. Similarly, VIP circulating levels were significantly increased in patients with SSc (p<0.001), whereas the increase of NPY levels did not reach statistical significance. However, both neuropeptides, following the same trend as NGF, increased as the disease worsened (skin score and lung disease). CONCLUSIONS: The increase of NGF and VIP in patients with SSc, the former in the diffuse subset of the disease, and in patients with prominent articular disease, may suggest a link between neurotransmitters and the disease pathogenesis. Neuropeptide circulating levels seem to increase only in patients with the most severe disease.

Biomarkers↗
PubMed2000

Reliability and validity of the Arthritis Hand Function Test in adults with systemic sclerosis (scleroderma).

OBJECTIVE: To determine the interrater and test-retest reliability and validity of the Arthritis Hand Function Test (AHFT) in persons with systemic sclerosis. METHODS: Interrater reliability of the AHFT was established by two raters independently scoring the performances of 20 women with systemic sclerosis. The same group of subjects was tested again 7-10 days later to determine test-retest reliability. Concurrent validity was established by the subjects' self-reports of their abilities to perform activities of daily living as measured by the Health Assessment Questionnaire and the Arthritis Impact Measurement Scales 2 (AIMS2). RESULTS: All of the items had excellent interrater intraclass correlation coefficients (ICC = 0.99-1.00). The ICCs for test-retest reliability were in the excellent (ICC = 0.80-0.97) range for most of the items and moderate (ICC = 0.57-0.73) for the others. Most of the items were moderately correlated with items on the AIMS2 (r = 0.45-0.69). CONCLUSION: The results from this study suggest that the AHFT is a reliable and valid test to measure hand function in persons with systemic sclerosis.

Activities of Daily Living↗
PubMed1980

Ultrastructural differences of interdigitating cells in human lymph nodes.

Eleven axillary lymph nodes from patients with different cutaneous disorders (systemic scleroderma, atopic eczema, psoriasis, hairy cell erythroderma, dermatopathic lymphadenitis) were examined by electron microscopy. In systemic scleroderma interdigitating cells (IDC's) showed typical ultrastructural features as well as intimate contacts with neighboring lymphocytes. In atopic eczema IDC's were characterized by widespread invaginations of the cell membrane, and an increase in tubulo-vesicular structures and microfilaments. Similar observations have been made in dermatopathic lymphadenitis. In psoriasis and hairy cell erythroderma. IDC's showed only a few interdigitations and invaginations of the cell surface. It is supposed that these structural changes in IDC's reflect the different immunological conditions of the diverse cutaneous disorders.

Dermatitis, Atopic↗
PubMed1995

HLA class II genes associated with anticentromere antibody in Japanese patients with systemic sclerosis (scleroderma).

OBJECTIVE: To define further HLA class II gene associations with anticentromere antibody (ACA), a major serum antinuclear antibody in patients with systemic sclerosis (SSc). METHODS: HLA class II genes were determined using polymerase chain reaction/restriction fragment length polymorphisms in 94 Japanese patients with SSc (22 ACA positive and 72 ACA negative) and 50 race matched normal control subjects. RESULTS: Frequency of DQB1*0501 was increased in ACA positive SSc patients compared with ACA negative SSc patients (36% versus 13%; p = 0.02, odds ratio = 4.0, 95% confidence interval 1.1 to 13.9), but the association of ACA with a polar amino acid at position 26 in the DQB1 beta 1 domain, which was demonstrated in white North Americans, was not observed in Japanese. The DRB1*0101, *0405, and *1302 alleles were associated with high ACA titres, whereas DRB1*1502 was associated with low ACA titres and a low frequency of centromere protein C (CENP-C) reactivity. CONCLUSIONS: These results suggest that the ACA response is associated with multiple HLA class II genes and that ACA positive SSc patients are heterogeneous in terms of immunogenetic background.

Alleles↗
PubMed1998

QT interval and QT dispersion in systemic sclerosis (scleroderma).

OBJECTIVES: To measure QT interval and QT dispersion, and to evaluate possible relationships between these measurements, heart rate variability parameters, and early myocardial involvement in patients with systemic sclerosis (SSc). DESIGN: Prospective study. SETTING: Tertiary care centre, University 'La Sapienza', Rome, Italy. SUBJECTS: Thirty-eight patients with SSc (35 females and three males, mean age 47 +/- 11 years), 19 patients with the diffuse form of disease and 19 with the limited form, and 17 healthy controls (11 females and six males, mean age 43 +/- 10 years) were studied. INTERVENTIONS: Both patients and control subjects underwent resting 12-lead electrocardiogram and 24-hour Holter monitoring. Moreover, resting myocardial scintigraphy with 99 m Tc-sestamibi was performed in all SSc patients. MAIN OUTCOME MEASURES: Bazett's formula was used to obtain rate corrected value of QT interval (QTc). QT and QTc dispersion were defined as the difference between maximum and minimum QT or QTc interval, respectively. Twenty-four-hour heart rate variability was analysed both in the frequency and in the time domain. RESULTS: Twenty-three SSc patients (60.5%) had myocardial resting perfusion defects (group A) and 15 (39.5%) did not (group B). Maximum QTc interval, QT and QTc dispersion were significantly increased in SSc patients compared to the control subjects. No significant differences between group A and group B were observed for all QT measurements. Furthermore, maximum QTc interval, QT dispersion and QTc dispersion were significantly increased in group A patients compared to the control group. Total power, low-frequency, and high-frequency values were significantly lower in all SSc patients, whether in group A or group B, than in control subjects. On the other hand, low-frequency/high-frequency ratio was similar in all groups. Heart rate variability in time domain analysis showed no statistically significant differences between groups. CONCLUSIONS: Patients with SSc have increased QTc interval, QT dispersion, and QTc dispersion. The role of autonomic nervous system and myocardial involvement on ventricular repolarization in patients with SSc needs further investigation.

Adult↗