Scleroderma and primary biliary cirrhosis.

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

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 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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    ABSTRACT: INTRODUCTION: to determine the frequency of hepatic disease in patients with scleroderma and, secondarily, to investigate the frequency of hepatitis B and C virus infection and determine the frequency of autoantibodies in this disease. MATERIAL AND METHODS: patients with scleroderma followed at Hospital Santa Izabel were included in the study and patients with acne vulgaris served as a comparison group. RESULTS: considering the 65 scleroderma patients, 35% had elevated gamma-glutamyltranspeptidase (gamma-GT), 30% had elevated alkaline phosphatase and 17.1% had alanine aminotransferase (ALT) higher than the reference range. Raised ALT levels were more common in the scleroderma patients than in the control group. Nineteen percent (19%) of the patients tested positive for anti-smooth muscle antibodies and only one patient had anti-mitocondria antibodies. There was no statistical difference between the two groups regarding antibody testing. Anti-HCV antibodies were observed in one patient and HBsAg was detected in another scleroderma patient. There was no patient with clinically significant hepatic disease. CONCLUSIONS: the present study showed that clinical hepatic disease did not occur in our scleroderma patients, despite the relatively increased frequency of liver enzymes abnormalities.
    Revista Brasileira de Reumatologia 10/2007; 47(5):330-333. · 0.86 Impact Factor
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    ABSTRACT: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by immune-mediated destruction of the small and medium size intrahepatic bile ducts. PBC patients often have concomitant autoimmune diseases, which are most often autoimmune thyroid disease, as well as Sicca syndrome. Occasionally, some PBC patients will also have systemic sclerosis of the limited cutaneous type (lcSSc). Conversely, up to one-fourth of SSc patients are positive for antimitochondrial antibody, the serologic hallmark of PBC. It is also common for SSc patients to have concomitant autoimmune disease, which may include PBC in rare cases. This has led to speculation of shared environmental and/or genetic factors, which lead to the development of PBC in SSc patients and vice versa. Recent genetic studies have revealed associations with several genes in both SSc and PBC. PTPN22 is one gene that has been associated with SSc, but not with PBC. It may be argued that some SSc patients with a particular genotype, which shares genes found in both conditions may develop PBC. Likewise, particular genes such as PTPN22 may infer susceptibility to SSc alone. The presence of PTPN22 may also contribute to the development of SSc in PBC patients. The lack of a large number of overlapping genes may, in part, explain the relative rarity of PBC with SSc and vice versa. This review will examine the literature surrounding the genetic associations of PBC and SSc, and the role of PTPN22 in particular.
    Autoimmunity Highlights. 3(1).
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    ABSTRACT: Primary biliary cirrhosis (PBC) can be complicated by systemic sclerosis (SSc) and more specifically, limited cutaneous SSc (lcSSc), which was previously called CREST syndrome. Moreover, combined PBC and SSc has been described in many case reports. Although neither the etiology of PBC nor that of SSc has been elucidated, some genetic and immunological factors are known to be shared. Both disorders are autoimmune fibrotic diseases characterized by increased levels of pro-fibrotic cytokines transforming growth factor β (TGFβ) and IL-6, which have recently been suggested to influence Th17 cells and regulatory T cells involved in acquired immunity. LcSSc is accompanied by CREST symptoms, although complete CREST cases are rare, with relatively high prevalence of Raynaud's phenomenon, sclerodactyly and telangiectasia, and lower prevalence of calcinosis and esophageal dysmotility. Because patients with anti-centromere antibody-positive PBC-SSc are at a high risk of developing portal hypertension, particular attention should be paid to the management of gastroesophageal varices. In addition, the management of SSc-related non-hepatic disorders, such as pulmonary fibrosis, pulmonary hypertension, heart disorder, infection and malignancy, is also important for improved outcomes. Since PBC is often complicated by rheumatic disease, hepatologists should keep the possibility of systemic disorder in mind when examining PBC patients.
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