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Results for “Scleroderma, Localized”

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PubMed1994

Scleroderma renal crisis with minimal skin involvement and no serologic evidence of systemic sclerosis.

We describe a 75-year-old male patient who developed end-stage renal failure secondary to scleroderma renal crisis. The patient had cutaneous involvement limited to his fingers and feet, and had repeatedly negative serologic tests for antinuclear antibodies. This case illustrates that scleroderma renal crisis should be considered in the differential diagnosis of unexplained acute renal failure in patients with suspected systemic sclerosis, even if they lack the typical extensive cutaneous changes and the expected serologic findings.

Aged↗
PubMed2001

Elevated serum levels of soluble L-selectin in patients with systemic sclerosis declined after intravenous injection of lipo-prostaglandin E1.

To determine whether serum soluble L-selectin (sL-selectin) levels are elevated in patients with systemic sclerosis (SSc) and whether serum sL-selectin levels change after treatment with lipo-prostaglandin E1 (lipo-PGE1), serum sL-selectin levels were examined by ELISA in patients with SSc (n=24), psoriasis vulgaris (n=22), and contact dermatitis (n=9), as well as normal control subjects (n=26). In five patients with SSc, serum sL-selectin levels were examined before and after intravenous injections of lipo-PGE1. Serum sL-selectin levels were significantly increased in patients with SSc (P<0.01) than those in normal control subjects. The elevated serum sL-selectin levels in patients with SSc significantly decreased 1 day (P<0.05), 7 days (P<0.05) and 14 days (P<0.05) after starting the treatment with lipo-PGE1. These results suggest that L-selectin may be involved in the disease process in SSc, and lipo-PGE1 may alter some inflammatory events in SSc.

Adolescent↗
PubMed1995

Scleroderma.

In practice, the classification of scleroderma is less problematic than that of most other connective tissue diseases given the distinctive pattern of skin involvement. Classification of early and limited disease is more problematic and is difficult to separate from severe forms of Raynaud's phenomenon. Division of scleroderma into two groups depending on the presence or absence, early in the disease, of truncal skin involvement makes biological and clinical sense. Measurement using a clinical skin score remains the best approach to monitoring disease progress.

Humans↗
PubMed1996

Lung involvement in systemic sclerosis (scleroderma): relation to classification based on extent of skin involvement or autoantibody status.

Lung involvement accounts for significant morbidity and is a leading cause of mortality in patients with systemic sclerosis (SSc). It has been shown that different patterns of pulmonary involvement are seen in different subtypes of SSc. This paper reports a retrospective review of 72 patients with SSc to determine whether disease classification according to the extent of skin involvement alone (diffuse vs. limited) or autoantibody status was predictive of pulmonary parenchymal involvement. The diagnosis of interstitial lung disease was based on pulmonary function tests and chest radiographs. Restrictive lung disease was common in both limited SSc (lSSc) and diffuse SSc (dSSc), occurring in 30% and 50% of these patients respectively (P = 0.16). Radiographic evidence of significant interstitial disease was also comparable between the groups [nine of 32 lSSc patients (28%) vs. six of 17 dSSc patients (32%), P = n.s.]. No significant difference in mean lung function was found between patients with anti-Scl 70 antibody (n = 12) compared to those without (n = 60) (TLC 79.0 +/- SE 5.1% predicted vs. 82.8 +/- 2.2, P = n.s.; DLCO 63.0 +/- 5.1 vs. 59.7 +/- 2.5, P = n.s.). By contrast, statistically significant differences in mean lung function were found between patients with anticentromere antibody (ACA) (n = 24) and those without ACA (n = 48) (TLC 98.6 +/- SE 3.9% predicted vs. 79.7 +/- 3.1%, P < 0.001); and less frequent radiographic evidence of severe interstitial disease (0 of 17 with significant interstitial changes on chest radiograph vs. 15 of 32 (47%), P = 0.002). It is concluded that classification of SSc patients on the basis of the distribution of skin involvement poorly predicts the occurrence of interstitial lung disease. On the other hand, ACA is highly associated with the absence of interstitial lung disease.

Adult↗
PubMed1998

Immunologic abnormalities in scleroderma.

The immune system abnormalities of scleroderma are diverse and in association with vascular and mesenchymal cell abnormalities, contribute to the organ system dysfunction and clinical expression of the disease. Recent insights into novel immune regulatory pathways, immune reactivity to self-antigens, and the potential association of persistent fetal cells and disease expression may serve to increase our understanding of this enigmatic disease.

Animals↗
PubMed2006

[Systemic sclerosis and cancer].

The combination of skin induration with clinical features such as dyspnea, facial telangiectasia, digital infarctions and/or dysphagia supports the diagnosis of systemic sclerosis. The oesophageal dysmotility is associated with symptoms which may delay the diagnosis of gastro-oesophageal cancer. Herein we report a case of long standing systemic sclerosis with heartburn and dysphagia symptoms which were monitored closely. Unfortunately, these symptoms delayed the diagnosis of gastric adenocarcinoma. This case prompted us to review the evidence of the association of cancer and systemic sclerosis and if any oncologic evaluation is required during the follow-up of patients affected with systemic sclerosis.

Adenocarcinoma↗
PubMed2004

Hepatocyte growth factor both prevents and ameliorates the symptoms of dermal sclerosis in a mouse model of scleroderma.

Systemic sclerosis (SSc) is a connective tissue disorder with an unknown etiology. There are currently no effective therapies for SSc. (In this study, working with a bleomycin(BLM)-induced scleroderma model mice, we performed two transfections of human hepatocyte growth factor (HGF) cDNA into the skeletal muscle and showed that this treatment not only helped to prevent the dermal sclerosis simultaneously injected BLM but also improved the symptoms of dermal sclerosis induced by BLM 4 weeks previously.) RT-PCR, ELISA and an immunohistochemical analysis revealed that both mRNA and protein of human HGF as well as murine HGF were enhanced in the skin, lung, muscle and the serum after two transfections of human HGF cDNA. These analyses also revealed that this treatment significantly reduced both the expression of the TGF-beta1 mRNA and the production of TGF-beta1 on macrophage-like cells that infiltrated the dermis and the fibroblastic cells in BLM-induced scleroderma. Furthermore, HGF-gene transfection both prevented and ameliorated the symptoms of not only dermal sclerosis but also of lung fibrosis induced by a subcutaneous BLM injection. These results indicated that gene therapy by the transfection of the human HGF cDNA may thus be a useful therapy for SSc and lung fibrosis involved with SSc.

Animals↗