Scleroderma and Primary Biliary Cirrhosis
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TLDR
Two cases of scleroderma and primary biliary cirrhosis are described and it is suggested that the association may be due to a common “autoimmune” process.Abstract:
Two cases of scleroderma and primary biliary cirrhosis are described One had systemic sclerosis with primary biliary cirrhosis of six years' duration at the stage of ductular proliferation The other had the CRST syndrome (calcinosis, Raynaud's phenomenon, sclerodactyly, and telangiectases) with primary biliary cirrhosis at the florid stage Several similar cases were found in a review of other reports, and it is suggested that the association may be due to a common "autoimmune" processread more
Citations
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Aberrant expression of HLA-DR antigens on bileduct epithelium in primary biliary cirrhosis: relevance to pathogenesis.
TL;DR: The increased HLA-A,B,C, expression may be a means of amplifying T-cell cytotoxic responses in PBC and enables these cells to present "self antigens" to sensitised T-lymphocytes and to promote autorecognition.
Journal ArticleDOI
Cellular infiltrates in scleroderma skin.
TL;DR: Cellular infiltrates, perivascular or diffuse, were noted in 49% of SS and 84% of LS patients and consisted of lymphocytes, plasma cells, and macrophages; no correlation was noted between the presence or severity of skin cellular infiltrates and serum serologic abnormalities.
Journal ArticleDOI
Clinical pattern and course of disease in primary biliary cirrhosis based on an analysis of 236 patients
Erik Christensen,John Crowe,Deborah Doniach,Hans Popper,Leo Ranek,Juan Rodés,Niels Tygstrup,Roger Williams +7 more
TL;DR: In analysis of subgroups, age, histologic stage, and particularly the serum bilirubin level were important prognostic factors and the mitochondrial antibody titer showed a high rate of progression with time.
Journal ArticleDOI
Positive antimitochondrial antibody but normal alkaline phosphatase: Is this primary biliary cirrhosis?
H.C. Mitchison,Margaret F. Bassendine,Alex Hendrick,Mark K. Bennett,Graham Bird,Alexander J. Watson,Oliver F. W. James +6 more
TL;DR: It is concluded that the finding of an antimitochondrial antibody titer ≥1/40 is strongly suggestive of primary biliary cirrhosis even in the absence of symptoms and the presence of a normal alkaline phosphatase.
Journal ArticleDOI
Primary Biliary Cirrhosis: A Model Autoimmune Disease
TL;DR: Better understanding of the pathogenesis of primary biliary cirrhosis is needed to develop specific immunotherapies and treatments to arrest the disease's progress have included corticosteroids, azathioprine, cyclosporin, and D-penicillamine.
References
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Journal ArticleDOI
Systemic scleroderma, A clinical study of 727 cases.
TL;DR: A longterm clinical study of scleroderma is believed to be unique in the extremely large number of patients observed, and to add to knowledge of a poorly understood entity.
Journal Article
Tissue antibodies in primary biliary cirrhosis, active chronic (lupoid) hepatitis, cryptogenic cirrhosis and other liver diseases and their clinical implications.
TL;DR: The findings suggest that `M' antibodies do not arise merely in response to liver damage and confirm the value of the test for the differential diagnosis between primary biliary cirrhosis and extrahepatic biliary obstruction although care must be taken in interpretation of the results in cases with associated connective tissue disorders where a significant incidence of positive reactions was observed.
Journal Article
Primary biliary cirrhosis. chronic non-suppurative destructive cholangitis.
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Progressive Systemic Sclerosis
Harper Ra,Jackson Dc +1 more
TL;DR: It has been found that over 50 per cent of the patients had positive radiological changes in the colon that are specific for scleroderma and consist of wide-necked pseudo diverticula in the transverse and descending colon.
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