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)

Antinuclear and/or antinucleolar antibodies were demonstrated in the sera of 74 of 76 patients (97%) with progressive systemic sclerosis, using tissue culture cells (HEp-2) as substrate in the indirect immunofluorescent method. Six patterns of nuclear staining and three nucleolar patterns were recognized. The nuclear patterns were centromere, fine speckles, coarse speckles, diffusely grainy, homogeneous and nuclear dots. The nucleolar patterns were speckled, homogeneous and clumpy. The results of digestion studies with ribonuclease, deoxyribonuclease and trypsin suggested that the nuclear antigens are proteins, some of which may be associated with chromatin. The nucleolar antigens appeared to be nucleic acid in nature. Certain characteristic serologic and clinical features associated with staining patterns were observed. The diffusely grainy pattern was seen only in sera containing antibody to Scl-70 antigen. Centromere staining was confirmed to be highly selective for the CREST (Calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly and telangiectasis) variant of progressive systemic sclerosis with rheumatoid factor titres higher in these patients with anti-centromere antibodies.
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PMID:Association of antinuclear and antinucleolar antibodies in progressive systemic sclerosis. 704 33

A series of patients with fatal progressive systemic sclerosis was reviewed with regard to pathologic, clinical, and serologic evidence of thyroid disease. Histologic evidence of severe fibrosis of the thyroid gland was found significantly more frequently in 56 progressive systemic sclerosis cases (14%) compared to an age and sex matched control autopsy series (2%) from the same institution. Based on determination of serum free thyroxine, free triiodothyroxine (T3), thyroid stimulating hormone, and reverse T3, 27 patients were classified as euthyroid (11), euthyroid sick (9), and hypothyroid (7). Patients with hypothyroidism more frequently had subcutaneous calcinosis. Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and multiple telangiectasias (the CREST syndrome variant of progressive systemic sclerosis); all thyroid glands from the hypothyroid patients had fibrosis, but lymphocytic infiltration was an infrequent finding. Six hypothyroid patients had high titers of serum antithyroid antibodies, suggesting autoimmune thyroid disease. Thyroid gland fibrosis and hypothyroidism, possibly of autoimmune pathogenesis, are thus frequent and often unsuspected findings in progressive systemic sclerosis.
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PMID:Thyroid disease in progressive systemic sclerosis: increased frequency of glandular fibrosis and hypothyroidism. 728 93

A multicenter, ongoing study of early-diagnosed cases of systemic sclerosis and comparison patients with systemic lupus erythematosus, polymyositis/dermatomyositis, and Raynaud's phenomenon was conducted in order to develop classification criteria for systemic sclerosis. Preliminary criteria are proposed namely, the finding of either the sole major criterion, i.e., proximal scleroderma, or two or more of the minor criteria, i.e., 1) sclerodactyly, 2) digital pitting scars of fingertips or loss of substance of the distal finger pad, and 3) bilateral basilar pulmonary fibrosis. When applied to the case and comparison patients included in this study, these proposed criteria had a 97% sensitivity for definite systemic sclerosis and 98% specificity.
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PMID:Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. 737 88

Gastrointestinal tract involvement in progressive systemic sclerosis (PSS) is common. A 46-year-old woman with PSS and calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasias with evidence of widespread intestinal tract involvement is described in whom multiple colonic telangiectasias (angiodysplasias) were found on colonoscopy, a finding not previously reported to our knowledge. The telangiectasias represent a potential cause of gastrointestinal tract bleeding.
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PMID:Colonic telangiectasias in scleroderma. 739 24

Autoantibodies specific against fibrillarin, a 34-kD nucleolar protein associated with U3-snRNP, are present in patients with systemic sclerosis (SSc). To understand the mechanisms involved in the induction of these autoantibodies, we prepared a series of human fibrillarin recombinant proteins covering the entire molecule and analyzed their interaction with the autoantibodies present in various connective tissue diseases. Our results showed that antifibrillarin autoantibodies are present not only in SSc, as previously reported, but also in a variety of other connective tissue diseases. Patients with SSc (58%), mixed connective tissue diseases (60%), CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dismotility, sclerodactyly, and telangiectasia syndrome) (58%), systemic lupus erythematosus (39%), rheumatoid arthritis (60%), and Sjogern's syndrome (84%) showed presence of antifibrillarin autoantibodies. Results obtained from competitive inhibition radioimmunoassay and Western blot analyses with purified recombinant fusion proteins revealed that these autoantibodies react primarily with epitope(s) present in the NH2- (AA 1-80) and COOH-terminal (AA 276-321) domains of fibrillarin. Autoantibodies reacting with internal regions of fibrillarin are less frequent. Analysis of the hydrophilicity profiles of reactive peptides showed presence of three potential antigenic sites in the NH2- and two in the COOH-terminal regions. While a hexapeptide sequence NH2 terminus of fibrillarin is shared with an Epstein-Barr virus-encoded nuclear antigen, the COOH-terminal region shares sequence homology with P40, the capsid protein encoded by herpes virus type 1. Interestingly, these two regions of fibrillarin also contain the most immunodominant sequences, as predicted by surface probability and the Jameson and Wolf antigenic index. These observations suggest that molecular mimicry might play an important role in the induction of antifibrillarin autoantibodies.
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PMID:Antifibrillarin autoantibodies present in systemic sclerosis and other connective tissue diseases interact with similar epitopes. 753 74

A 59-year-old man was admitted to hospital with sclerodactyly and high grade fever. He complained of wrist joint pain and swelling of bilateral hands. Despite of administration of non steroid anti-inflammatory drug and steroid the symptoms were progressive and sclerodactyly spreaded to the forearm. Through the results of the laboratory examination, he was diagnosed as progressive systemic sclerosis (PSS). Chest CT scan showed an anterior mediastinal tumor, and histological examination revealed a spindle cell type thymoma with mild lymphocytic infiltration. During hospitalization, the pulse therapy of steroid was administrated. Progressive sclerodactyly and interstitial pneumonia were improved, but stiffness of hand and joint pain was not diminished. In expectation of improvement of the symptoms, mediastinal tumor was removed. Histological diagnosis was thymoma of epitherial type. After surgery, symptoms decreased and at last diminished, dosage of steroid could be tapered.
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PMID:[Thymoma associated with progressive systemic sclerosis--a case report]. 788 54

Progressive systemic sclerosis (PSS) is a connective tissue disease characterized by fibrosis and thickness of cutis and subcutis (scleroderma) and deterioration of small arteries and capillary vessels, with changeable visceral renal, cardiac, intestinal and pulmonary involvements. The disease is characterized by cutaneous sclerosis, that is to say by the increase of consistence and thickness of cutis that lose her usual elasticity. The sclerosis can be limited to the fingers (sclerodactyly) or can involve otherwise (acrosclerosis); many other time is diffuse also to upper limbs and to thorax (diffuse scleroderma). The disease is rare and its incidence is variable from 2 to 10 x 10(6); more frequently hits women than men in the ratio of 3 to 1 or 8 to 1 (according to certain Authors). In the elderly is more frequently represented by the CREST syndrome (calcinosis, Raynaud's phenomenon, oesophageal dismotility, sclerodactyly, teleangiectasia). The authors have considered nine patients with pulmonary involvement of progressive systemic sclerosis: five with CREST syndrome and four with diffuse scleroderma. In addition to the common routine examinations, we performed chest radiography (CXR), pulmonary function tests, fiberoptic bronchoscopy and bronchoalveolar lavage (BAL), Gallium 67 scanning and high resolution computed tomography (HRCT) with the purpose to consider the role of these medical examinations in diagnosis and the follow-up of pulmonary involvement in scleroderma. From the results we have observed that: 1) in early disease chest radiography may be silent even if the patient shows a restrictive syndrome and reduction of diffusion capacity of carbon monoxide (DLCO); 2) chest radiography, pulmonary function tests and Gallium 67 scanning have traditionally been used to measure the degree of lung involvement in SSP but unfortunately, none of these reliably predicts disease activity or future course; 3) bronchoalveolar lavage shows the degree of lung inflammation (alveolitis) characterized by the increase of total BAL cell counts and by higher neutrophil cells in the initial stage of the disease; 4) even HRCT shows in this stage, the typical aspect of higher endoalveolar cellularity with ground glass appearance. With the progression of anatomical damage, there can be found even appearance of complete destruction of pulmonary architecture with the typical alteration of honeycomb. These findings support the hypothesis that a ground glass appearance on HRCT is the radiographic equivalent of alveolitis found in BAL. Furthermore HRCT shows itself useful in the follow-up the disease and in detecting of its activity condition.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Pulmonary involvement in sclerodermia]. 808 31

The concentrations of sCD4 and sCD8 in 69 patients with systemic sclerosis (SSc) were examined by using a sandwich enzyme-linked immunosorbent assay. The patients with SSc had significantly higher concentrations of sCD8 (mean 249.2 U/ml (SD 155.1), median 224 U/ml) than the normal subjects (mean 149.3 U/ml (SD 42.1), median 148 U/ml). The concentration of sCD4 in patients with SSc were significantly lower (mean 6.2 U/ml (SD 3.8), median 5.0 U/ml) than in the normal subjects (mean 10.9 U/ml (SD 4.1), median 10.3 U/ml). The concentration of sCD8 in patients with diffuse sclerosis tended to be higher than in those with sclerodactyly.
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PMID:Soluble CD4 and CD8 molecules in patients with systemic sclerosis. 812 11

We report the case of a 67 years old woman diagnosed of CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasias) variant of generalized scleroderma, that suffered a Brucella endocarditis on mitral valve affected by the primary disease. We examine the cardiac lesions in progressive systemic sclerosis, pointing up the rare involvement of the valves, together with the evolution and treatment of Brucella endocarditis, uncommon entity.
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PMID:[Brucella infective endocarditis on sclerodermic mitral valve. Report of a case]. 823 16

The association of cancer and connective tissue disease is well known, the most frequent being certainly with dermatomyositis. The association cancer and PSS is more controversial. The incidence of neoplasia in that group seems to be comparable with the general population but the proportion of certain types of cancer is different, and the temporal relationship with the apparition of symptoms of PSS is stunning. The hypothesis actually in favor is an imbalance of the immune system, which cause the diminution of the immune surveillance and the apparition of cancer and a concomitant dysregulation of the system, causing the fibrosis of the PSS. We describe a 75 year-old white female who developed a colic adenocarcinoma; she also had, concomitantly, a systemic scleroderma, with sclerodactyly and pulmonary fibrosis. The patient corresponded to the criteria of the American Rheumatism Association for progressive systemic scleroderma (PSS). The prognosis of patients with PSS depends on their systemic involvement but also, we believe, in the more aged group, on the apparition of a neoplasia.
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PMID:[Colonic adenocarcinoma and scleroderma]. 823 78


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