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)

Sharp syndrome (mixed connective tissue disease) is a distinct rheumatic syndrome with symptoms of various connective tissue diseases (rheumatoid arthritis, systemic lupus erythematodes, progressive systemic sclerosis, polymyositis and others). 15 patients with mixed connective tissue disease are described. The clinical picture and diagnostic criteria are evaluated and the course of the disease, treatment and prognosis are discussed.
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PMID:[Sharp's syndrome (mixed connective tissue disease). Clinical aspects diagnosis and prognosis]. 19 98

In contrast to the 15-20% incidence of the coexistence of acute dermatomyositis-polymyositis and malignancy, it has been accepted traditionally that the association of progressive systemic sclerosis, a disease with several features that may overlap the former condition, and malignancy is purely fortuitous. This experience has not been altered by the material presented in this review. However, the factual coexistence has been illuminated by a review of the pertinent literature and presentation of 12 previously unpublished case reports. Four cases concern pulmonary malignancies in PSS; eight are of an associated non-pulmonary malignancy. In the first group, the development of a malignancy superimposed on the chronic fibrosing changes in the lungs of PSS does not seem so strange, particularly in view of a possible immunologic reaction by collagen in considering pathogenesis. This immunologic process might be similar to a related immunologic process responsible for the development of malignant cells in pulmonary and other tissue, where normal cells usually are found. The high incidence of males is related to the high incidence of males in Veterans Administration Hospitals, the principal population source of these cases.
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PMID:Progressive systemic sclerosis (PSS) and malignancy, pulmonary and non-pulmonary. 21 19

Mixed connective tissue disease is a clinical entity defined by overlapping features of progressive systemic sclerosis, systemic lupus erythematosus, polymyositis, rheumatoid arthritis, and distinct serologic findings. Esophageal dilatation and dysmotility have been the only gastrointestinal manifestations reported. Three patients with serologic findings of mixed connective tissue disease and extensive gastrointestinal involvement compatible with the changes found in progressive systemic sclerosis are presented. Gastrointestinal manifestations of progressive systemic sclerosis are reviewed and were found to be indistinguishable from the findings in these patients.
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PMID:Gastrointestinal systemic sclerosis in serologic mixed connective tissue disease. 30 6

Antibodies to ribonucleoprotein (RNP) were detected by an immunofluorescence technique based on the sensitivity of speckled antinuclear antibodies to ribonuclease. These antibodies were found to identify a group of patients with a consistent set of clinical features, especially arthritis, swollen hands, Raynaud's phenomenon, and myositis. The presence of anti-RNP antibodies in sera from patients with polymyositis, systemic lupus erythematosus, and systemic sclerosis was also associated with these clinical features. Other studies of the clinical significance of these antibodies support the concept that they appear to identify a group of patients with a distinct clinical condition.
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PMID:Clinical significance of antibodies to ribonucleoprotein. 31 58

The treatment of the Goodpasture syndrome, collagenoses (rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis/scleroderma, matosus, progressive systemic sclerosis/scleroderma, dermatomyositis/polymyositis), necrotizing angiitis (periarteritis nodosa, Wegener's granulomatosis) and idiopathic pulmonary fibrosis is reviewed. Their pathogenesis and morbid anatomy is outlined. The uncommunness of these disorders and their pulmonary manifestations is emphasized. The most frequently employed therapeutic agent are the corticosteroids together with acetylsalicylic acid and indomethacin. The effectiveness of immuno-suppressive and cytostatic drugs (azothioprine, cyclophosphamide, chlorambucil) and of D-penicillamine in the treatment of the different disorders is described. Standard programmes for treating any of these diseases have not yet emerged; the long-term results tend do be unsatisfactory. Co-operation with other specialists (nephrologists, rheumatologists, dermatologists) is often necessary in the treatment of these disorders.
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PMID:[Treatment of auto-immune diseases with special reference to their pulmonary manifestations (author's transl)]. 37 47

Six patients with eosinophilic fasciitis are presented. This syndrome is characterized by indurative swellings of arms and legs, with rapidly progressing difficulties in extending elbows, wrists, and fingers, and often limited motion of shoulders and ankle joints. Pain when contracting muscles, and weakness of proximal muscles and hand grip are common features. The frequent occurrence of localized skin lesions has presented differential diagnostic difficulties to systemic sclerosis and to polymyositis. Visceral involvement and Raynaud's phenomena, however, are absent or mild. Blood eosinophilia, hypergammaglobulinemia, and unspecific signs of inflammation are found. Biopsy of muscle fascia gives characteristic histopathological findings of cell infiltrations (mostly mononuclear cells, frequently eosinophils) and vascular proliferation, in the middle layer of a thickened fascia. Skin changes are prevalent, but not conclusive for the diagnosis, and myositis in some patients might be difficult to distinguish from polymyositis. The importance of the clinical recognition of eosinophilic fasciitis and the inclusion of fascia in diagnostic muscle biopsies, is underlined.
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PMID:Eosinophilic fasciitis. Review and report of six cases. 53 17

Upon careful examination, 23 of 24 patients with progressive systemic sclerosis (PSS) were found to have abnormalities of muscle. Nineteen patients presented a homogenous pattern of muscle abnormalities, which untreated was associated with a stable course ("simple myopathy"). Three patients demonstrated inflammatory muscle disease indistinguishable from polymyositis while a fourth patient developed marked weakness associated with a generalized neuropathic process. Muscle enzymes, electromyogram, and muscle biopsy permitted distinction among the different muscle disorders, a distinction that could have prognostic and therapeutic importance.
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PMID:Muscle disease in progressive systemic sclerosis: diagnostic and therapeutic considerations. 62 95

Frequency and type of pulmonary and pleural involvement in collagen disease (rheumatoid arthritis, progressive systemic scleroderma, polymyositis-dermatomyositis and lupus erythematodes are analysed on the basis of literature and of own cases with particular regard to the roentgenographic appearance.
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PMID:[Lung and pleural involvement in collagen disease (author's transl)]. 84 23

Lymphocyte cytotoxicity for target cells is a method for evaluating specific lymphocyte stimulation. In vitro lymphocytotoxicity has been demonstrated in chronic ulcerative colitis and granulomatous colitis, polymyositis and dermatomyositis, systemic scleroderma, recurrent aphthous stomatitis, and periodontal disease. We have investigated lymphocytotoxicity in 36 patients with various oral inflammatory diseases using an automated cell-counting system. This investigation demonstrated in vitro lymphocytotoxicity for gingival epithelial target cells by aggressor lymphocytes harvested from patients with recurrent aphthous stomatitis and periodontal disease. This effect was not seen when the lymphocytes were harvested from normal subjects or from patients with other oral mucosal inflammatory diseases. The automated cell-counting system corresponded very well with the assay technique of exclusion of supravital dye.
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PMID:Lymphocyte-epithelial cell interactions in oral mucosal inflammatory diseases. 97 90

A new and distinct rheumatic disease with features of systemic lupus erythematosus (SLE), progressive systemic sclerosis and polymyositis is described. Typical symptons are Raynaud's syndrome, swollen hands and fingers, polyarthritis or polyarthralgia and myositis. Other symptoms are less common and include skin lesions, decreased pulmonary diffusing capacity, diminished esophageal motility, lymphadenopathy, and polyserositis. The diagnosis of mixed connective tissue disease (MCTD) can be established by demonstration of a high titer of antinuclear antibodies and antibodies against extractable nuclear antigen (anti-ENA). Both antibodies are directed against ribonuclease-sensitive antigen substrate, which permits differentiation of patients with MCTD from those with other rheumatic diseases. A relatively favourable prognosis and a good response to corticoid medication are further characteristics of this disease. Three personally observed patients with MCTD are described.
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PMID:[The Sharp syndrome ("mixed connective tissue disease")]. 108 43


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