Scleroderma and Primary Biliary Cirrhosis

British medical journal 09/1970; 3(5717):258-9. DOI: 10.1136/bmj.3.5717.258
Source: PubMed


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 C.R.S.T. 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" process.

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    • "Despite several similar reports over the years, liver disease has not been considered a significant feature of scleroderma, and larger studies have demonstrated that liver disease was more common in the control groups. The association of lcSSc and PBC was first described in 1970 with two cases of PBC and limited scleroderma (Murray-Lyon et al., 1970). A further six cases were also reported, and several other case reports have found an association between lcSSc and PBC. "

    Systemic Sclerosis - An Update on the Aberrant Immune System and Clinical Features, 02/2012; , ISBN: 978-953-307-869-4
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    ABSTRACT: A new syndrome is described consisting of chronic liver disease typical of primary biliary cirrhosis together with scleroderma, Raynaud's phenomenon, calcinosis cutis and telangiectasia. Six female patients had pruritus, jaundice and hepatomegaly with marked elevation of serum alkaline phosphatase activity and a positive test for serum mitochondrial antibody. Extrahepatic bile ducts were normal. In addition, they had telangiectasias, resembling those seen in the Rendu-Osler-Weber syndrome, on the finger pads and lips and occasionally on the mucosa of the upper gastrointestinal tract. The other features of the CRST syndrome (calcinosis, Raynaud's phenomenon and sclerodactyly) were present to a varying degree.The association of primary biliary cirrhosis with a form of scleroderma suggests an immunologic etiology for the liver disease.
    The American Journal of Medicine 04/1971; 50(3):302-12. DOI:10.1016/0002-9343(71)90218-X · 5.00 Impact Factor
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    ABSTRACT: Cell-mediated immunity has been investigated using the leucocyte migration test in 32 patients with chronic active hepatitis or primary biliary cirrhosis. Twenty-six (75%) showed reactivity to liver extract. At least two antigens are involved. Cell-mediated reactivity was confirmed in some cases using lymphocyte ;blast' transformation. There was no correlation with the presence of auto-antibodies.Cell-mediated immunity to parotid extract was shown in 10 cases (28%). This is in accord with the occurrence of the sicca syndrome in liver disease, which has been confirmed in this study.
    Gut 07/1972; 13(6):427-9. DOI:10.1136/gut.13.6.427 · 14.66 Impact Factor
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