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Results for “Scleroderma, Diffuse”

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At least 991 records · Page 55Linked to original sources
PubMed2002

Enhanced expression of the receptor for granulocyte macrophage colony stimulating factor on dermal fibroblasts from scleroderma patients.

OBJECTIVE: To elucidate events that initiate the involvement and stimulation of fibroblasts in systemic sclerosis (SSc). METHODS: We examined 15 patients with SSc diffuse form, 15 with CREST syndrome, and 5 healthy subjects. Cultured fibroblasts obtained from skin biopsies in SSc involved and non-involved areas and norrmal skin fibroblasts were cultured with different doses of granulocyte macrophage colony stimulating factor (GM-CSF) to study the effects of this factor on the expression of GM-CSF receptor (GM-CSFR) on fibroblast proliferation and cellular adhesion structures. RESULTS: Cultured fibroblasts obtained from biopsies of normal and SSc skin areas express GM-CSFR and such expression is increased in SSc fibroblasts. GM-CSF stimulation in vitro did not increase SSc fibroblast growth, in spite of a strongly increased expression of the GM-CSFR. The adhesion structures are always more abundant in SSc fibroblasts as compared to healthy cells and GM-CSF seems able to increase cell adhesion plaques. CONCLUSION: We suggest that shift of fibroblasts toward a more adhesive differentiated pattern, due to or accompanied by an increased expression of GM-CSFR, may be an important event in the pathogenesis of SSc.

Adult↗
PubMed1994

Progression of childhood linear scleroderma to fatal systemic sclerosis.

An 8-year-old girl presented with linear scleroderma, no evidence of systemic disease, and a negative antinuclear antibody (ANA) test. Over the next 12 months, she functioned normally. However, over the subsequent 5 months, she developed dyspnea, progressive pulmonary hypertension, a positive ANA test, and died 17 months after presentation. At autopsy, diffuse pulmonary interstitial fibrosis, small pulmonary arterial fibroplasia, tricuspid and mitral valve subendocardial fibrosis, and distal esophageal fibrosis were seen. Contrary to suggestions in the rheumatology literature, childhood linear scleroderma, even when ANA negative at presentation, may progress to fatal systemic sclerosis.

Antibodies, Antinuclear↗
PubMed1984

Interstitial lung disease in scleroderma. Analysis by bronchoalveolar lavage.

Interstitial pulmonary fibrosis is a common feature of scleroderma (systemic sclerosis) which may result in impairment of pulmonary function and may be a major determinant of morbidity and mortality. Clinicopathologic observations suggest that interstitial and alveolar inflammation may appear prior to fibrosis. Using the bronchoalveolar lavage (BAL) technique, we have characterized the nature of the inflammatory process in the lower respiratory tracts of 19 non-smoking scleroderma patients. Eleven of 19 patients (58%) had increased percentages of neutrophils and/or eosinophils in BAL fluid. Five of 10 patients (50%) had elevations of IgG in BAL fluid. The presence of neutrophils was associated with a decreased lung diffusing capacity for carbon monoxide (P less than 0.05) and with more advanced radiographic features of interstitial fibrosis in patients with disease of more than 1 year's duration. This study suggests that scleroderma lung involvement may be characterized by an inflammatory alveolitis and that the presence of such inflammation may relate to the severity of the pulmonary disease.

Adult↗
PubMed1995

Left ventricular wall motion abnormalities in 80 patients with systemic sclerosis.

Segmental analysis of left ventricular wall motion was performed in 80 patients with systemic sclerosis by echocardiography. Half of the cases showed normal wall motion. Eighteen of the 57 patients with limited cutaneous systemic sclerosis (31.6%) exhibited hypokinetic wall motion abnormalities, while five of the 23 cases with diffuse cutaneous systemic sclerosis (21.7%) had similar findings. The three cases showing akinesis also belonged to the limited scleroderma subgroup. Our findings show that hypokinetic wall motion abnormalities can be detected in a remarkably high proportion of cases with limited cutaneous systemic sclerosis.

Adult↗
PubMed2002

Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome.

Fibrosing alveolitis associated with systemic sclerosis (FASSc) has a better prognosis than idiopathic pulmonary fibrosis. In view of recent reports that idiopathic nonspecific interstitial pneumonia (NSIP) has a better prognosis than idiopathic usual interstitial pneumonia (UIP), we classified histologic appearances of surgical lung biopsies performed in 80 patients with FASSc. NSIP (n = 62, 77.5%), subcategorized as cellular NSIP (n = 15) and fibrotic NSIP (n = 47) was much more prevalent than UIP (n = 6), end-stage lung disease (ESL, n = 6), or other patterns (n = 6). There were 25 deaths (NSIP 16/62, 26%; UIP/ESL 6/12, 50%). Five-year survival differed little between NSIP (91%) and UIP/ESL (82%); mortality was associated with lower initial carbon monoxide diffusing capacity (DL(CO)) and FVC levels (p = 0.004 and p = 0.007, respectively). Survival and serial FVC and DL(CO) trends did not differ between cellular and fibrotic NSIP. Increased mortality in NSIP was associated with lower initial DL(CO) levels (p = 0.04), higher BAL eosinophil levels (p = 0.03), and deterioration in DL(CO) levels during the next 3 years (p < 0.005). We conclude that NSIP is the histopathologic pattern in most patients with FASSc. However, outcome is linked more strongly to disease severity at presentation and serial DL(CO) trends than to histopathologic findings.

Adult↗
PubMed1995

A review of autoimmune disorders anecdotally linked to mammary implants.

Autoimmune disorders lead to a sequence of tissue reactions and damage that may produce diffuse, systemic signs and symptoms. Litigants in many breast implants trials have claimed their autoimmune disorders (rheumatoid arthritis, scleroderma, and SLE) were from a causal relationship with mammary implants. Science suggests environmental factors, such as vinyl chloride, and hereditary factors may account for some of the prevalence of these disorders, however the exact cause remains a mystery and brings into question the claims being brought by litigants regarding implants.

Autoimmune Diseases↗
PubMedDate not supplied

Physical injury as a provoking factor in three patients with scleroderma.

A 51-year-old female developed linear-like scleroderma in the left thigh following a linear wound caused by a car accident. 27 years later she also developed a typical diffuse cutaneous systemic sclerosis with extensive skin involvement and bibasilar pulmonary fibrosis. The second case is a 39-year-old female who had a history of Raynaud's phenomenon since early childhood. She developed a morphea following a burning injury of the left thigh. 17 years later she also developed a typical limited cutaneous systemic sclerosis with sclerodactyly, skin ulcers and subcutaneous calcinosis. The third case is a 43-year-old female who developed a typical morphea of the right elbow around the site of a previous local corticosteroid injection. The two remarkable points of these 3 cases are the possible role of physical injury in the provocation of localized scleroderma and in the first 2 cases the unusual later development of a systemic form of scleroderma.

Adult↗
PubMed2004

Long-term evaluation of lung function in patients affected by scleroderma treated with cyclic iloprost infusions.

OBJECTIVE: The aim of this study was to evaluate pulmonary function in patients suffering from scleroderma treated for 3 years with cyclic iloprost infusions. METHODS: Thirty patients affected by scleroderma (five men and 25 women, mean age 49.3 years, mean disease duration 8 years, 19 with limited and 11 with diffuse disease patterns) were treated for 5 consecutive days every 4 months for 3 years with iloprost. Pulmonary function was evaluated at baseline and after 3 years. At the end of the trial, color Doppler echocardiography was performed in 26 of the patients. RESULTS: Spirometric parameters did not significantly change after 3 years of therapy when expressed as percentage of the predicted normal value. Diffusing lung capacity for carbon monoxide decreased at the limit of statistical significance. No patients developed clinical pictures suggestive of severe pulmonary arterial hypertension, which was confirmed in 26 subjects by evaluation of estimated pulmonary artery systolic pressure. CONCLUSIONS: This long-term study showed that lung function remained substantially stable after 3 years of treatment with cyclic iloprost infusions. No cases of severe pulmonary hypertension were observed.

Adult↗