Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036421 (PSS)
 10,989 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

As has previously been reported by many investigators, the anticentromere antibody is considered to be a useful serologic marker for the CREST (calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly and telangiectasia) variant of systemic sclerosis. However, this antibody also appears in other conditions. By screening antinuclear antibody tests using HEp-2 cells as substrate, 39 patients were shown to have the anticentromere antibody. These patients were divided into 4 groups: group 1 = 17 patients with systemic sclerosis; group 2 = 9 patients with Raynaud's phenomenon alone; group 3 = 7 patients with other connective-tissue diseases, and group 4 = 6 patients with conditions other than those present in groups 1-3. Follow-up over years revealed that some patients suffering solely from Raynaud's phenomenon (group 2) developed the symptoms of systemic sclerosis. In contrast, of the patients who did not have Raynaud's phenomenon (group 4), none developed any symptom suggesting systemic sclerosis. We suggest that the simultaneous presence of Raynaud's phenomenon and the anticentromere antibody predicts the occurrence of systemic sclerosis. In contrast, the presence of the anticentromere antibody alone cannot necessarily be used to predict the development of systemic sclerosis.
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PMID:Longitudinal study of patients with anticentromere antibody. 176 20

Blood lymphocytes from 37 patients with systemic sclerosis were characterised using monoclonal antibodies in a two colour flow cytometric (fluorescence activated cell sorter (FACS)) analysis. The ratio of helper CD4+ to suppressor/cytotoxic CD8+ T cells was raised in patients compared with that in 30 healthy controls owing to decreased CD8+ cells. In the patients CD4+ and CD8+ cells displayed an increased expression of the activation marker HLA-DR. The relative number of CD11b+ CD8+ lymphocytes (suppressor T cells) was normal, but the calculated absolute counts of this cell type were slightly reduced. The proportions and absolute numbers of suppressor inducer T cells, defined as CD45R+ CD4+ cells, were on average only half the levels observed in controls. These findings were not related to the inflammatory activity as measured by acute phase plasma proteins or serum immunoglobulins. Activated T cells were seen at all stages of the sclerotic process and especially during the early stages of the disease and in patients who had suffered occupational exposure to silica dust. A high proportion of activated T cells was also linked with impaired small intestine function but not with the degree of skin or lung involvement. A loss of suppressor inducer T cells was more pronounced later in the disease and in patients with the CREST (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) syndrome. These data provide further evidence for an involvement of T cell mediated immunity in the perpetuation of systemic sclerosis.
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PMID:Increase in activated T cells and reduction in suppressor inducer T cells in systemic sclerosis. 213 8

Muscle involvement was identified in 14 patients with scleroderma or a connective tissue disease overlap syndrome with predominant features of scleroderma. Patients presented with symmetrical proximal weakness indistinguishable from other inflammatory myopathies. Creatine kinase and electromyography were useful to demonstrate muscle involvement. Muscle histopathology demonstrated primarily the vasculopathy of scleroderma or polymyositis in similar numbers of patients. Scleroderma vasculopathy and polymyositis generally occur without specificity to diffuse scleroderma, the calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia syndrome, or an overlap syndrome with arthritis. Polymyositis also occurs when the vasculopathy of scleroderma involves other organ systems.
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PMID:Muscle involvement in the scleroderma syndromes. 224 71

Two patients who developed scleroderma after silicone breast augmentation are described. One patient presented with the classic features of systemic sclerosis, and the other with calcinosis, Raynaud's phenomenon oesophageal dysfunction, sclerodactyly, and telangiectasia (CREST syndrome). The connective tissue diseases associated with mammoplasty are reviewed.
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PMID:Scleroderma after silicone augmentation mammoplasty. A report of 2 cases. 230 Aug 58

Sera from 22 patients with progressive systemic sclerosis were tested for the ability to modify the angiogenic capability of normal human mononuclear cells. The sera from patients with acrosclerosis, including the abortive form (CREST syndrome: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), markedly enhanced this capability compared with sera from both healthy donors and patients with severe acrosclerosis and diffuse scleroderma. The enhancing effect of sera from patients with acrosclerosis decreased and/or disappeared in cases where the patient's acrosclerosis was chronic and severe. Thus, this test may be of diagnostic value in distinguishing various subgroups of systemic sclerosis.
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PMID:Modulatory effect of sera from scleroderma patients on lymphocyte-induced angiogenesis. 241 69

One hundred and forty five serum samples from patients with a connective tissue disease and 30 serum samples from healthy blood donors were analysed by immunoblotting. The presence of anti-Scl-70, which seems to discriminate between progressive systemic sclerosis (PSS) and the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) syndrome, was found in 31/64 (48%) patients with PSS, in 6/55 (11%) patients with systemic lupus erythematosus, in 2/26 (8%) patients with mixed connective tissue disease, and in none of 30 healthy blood donors. These data resulted in a specificity of 93% for this antibody in systemic sclerosis. For patients with PSS the duration of disease was significantly shorter in those with anti-Scl-70 antibodies than in those without, whereas the presence of anti-Scl-70 did not correlate with severity of disease. An 82% prevalence of anticentromere antibodies in patients with the CREST variant compared with a 4% prevalence in patients with PSS or with overlap syndrome confirms the high diagnostic value of this autoantibody for the CREST variant of PSS.
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PMID:Anti-Scl-70 antibodies detected by immunoblotting in progressive systemic sclerosis: specificity and clinical correlations. 251 13

The diagnostic and prognostic value of anti-Scl-70 autoantibodies in systemic scleroderma and other connective tissue diseases was investigated. A clinical and immunological study consisting of a search for anti-Scl-70 autoantibodies by immunodiffusion and immunoblot and a search for anti-centromere autoantibodies and antinuclear factors by indirect immunofluorescence was conducted in 57 cases of systemic scleroderma, 45 cases of CREST syndrome, 41 patients with suspected systemic scleroderma but who did not respond to the American rheumatism association (ARA) criteria, 35 cases of systemic lupus erythematosus, 8 cases of polymyositis, 40 cases of Raynaud's phenomenon and 48 controls. Anti-Scl-70 autoantibodies were found by immunodiffusion in 40 and by immunoblot in 45 cases of scleroderma with lesions proximal to the metacarpo-phalangeal joint (a major ARA criterion) and in 3 patients with suspected scleroderma but only one minor criterion: sclerodactyly. In systemic scleroderma with proximal lesions, the anti-Scl-70 autoantibody has a sensitivity of 0.85 and a specificity of 0.99. Anti-centromere autoantibodies were present in 39 cases of complete or incomplete CREST syndrome, 3 cases of Raynaud's phenomenon probably evolving towards a connective tissue disease and 1 case of systemic scleroderma with proximal lesions. The anti-Scl-70 and anti-centromere autoantibodies seem unable to coexist in the same patient and appear to be markers of two forms of scleroderma with different courses and prognoses. The Scl-70 antigen, which is the target of the anti-Scl-70 antibody, has been identified as topoisomerase 1, and a functional abnormality of this enzyme might contribute to some of the chromosomal abnormalities described in scleroderma.
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PMID:[Anti-Scl-70 and anti-centromere autoantibodies. Biological markers of 2 forms of systemic scleroderma]. 252 7

Case 1, a 49-year-old male who had been engaged in repair and reclamation of automobile tires, developed symptoms of vibration syndrome (Raynaud's phenomenon, numbness of both hands, tinnitus and impaired hearing) after some 30 years' use of a grinder and impact wrench. Two years thereafter, multiple sclerodermic lesions appeared over the trunk, upper extremities, and thighs; these disappeared about 2 years later. Histologically, hyperplasia and nodular swelling of collagen bundles were present in the dermis. An immunological study showed the serum to be positive for anti-centromere antibody, but no visceral lesions were demonstrable. This case corresponded to generalized morphea. Case 2, a 53-year-old male, developed symptoms of vibration syndrome (Raynaud's phenomenon, numbness of both hands, impaired hearing and arthralgia) after 25 years' use of a jack hammer in a quarry. Thereafter, sclerodermic changes of the forearms, lower legs, face and abdomen occurred with an associated sclerodactyly. Histological examination of involved skin revealed diffuse hyperplasia and homogenization of collagen bundles throughout the entire thickness of the dermis. These findings, together with serum positivity for anti-RNP antibody and dilation of the lower portion of the esophagus, led us to a diagnosis of progressive systemic sclerosis. We inferred that the vibration syndrome in the present cases might be related etiologically to these forms of scleroderma.
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PMID:[Two cases of scleroderma associated with vibration syndrome]. 254 58

A 37-year old man was suffered from Raynaud's phenomenon, sclerodactyly, and polyarthritis involving knees, shoulders, and hands. Recurrent skin ulcers were present in finger tips. Laboratory studies showed positive RA test, antinuclear antibody (nucleolar type), and anti-Scl 70 antibody. So diagnosis of progressive systemic sclerosis (PSS) was made. Progressed destructive arthritis with rheumatoid nodules developed in the patient. The joint destruction was severe and he was satisfied with the criteria of the American Rheumatism Association (ARA). This patient appears to be an overlapping case of PSS and rheumatoid arthritis (RA).
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PMID:[A case of progressive systemic sclerosis accompanied with rheumatoid arthritis]. 261 70

It is well known that patients with progressive systemic sclerosis (PSS), as well as other autoimmune disease, have various kinds of autoantibodies. We presented two PSS patients with anti Wa antibody, which had recently been reported as a new anti cytoplasmic antibody in only one case with PSS. Our first case, 49 years old female had clinical features of arthralgia, Raynaud's phenomenon, sclerodactyly, transient myalgia and sicca complex. Her laboratory findings showed hypergammaglobulinemia (1.9 g/dl), positive RAHA (1: 640) anti Wa antibody (1: 256), and anti SS-A antibody (1: 256). Second case, 64 years old female had also clinical features of Raynaud's phenomenon, sclerodactyly, dysphagia, dyspnea on exertion and dry mouth. Furthermore lung fibrosis and dysmobility of upper GI tract were observed. FANA (1: 20) was detected as nucleolar pattern, and the titer of anti Wa antibody was 1: 64. Clinical findings of myositis were not seen in both cases. This study suggested that anti Wa antibody might be one of the diagnostic marker on PSS.
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PMID:[Clinical study on two cases of progressive systemic sclerosis with anti Wa antibody]. 263 85


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