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Sex differences associated with primary biliary cirrhosis.

Smyk DS, Rigopoulou EI, Pares A, Billinis C, Burroughs AK, Muratori L, Invernizzi P, Bogdanos DP - Clin. Dev. Immunol. (2012)

Bottom Line: There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma.Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive.This paper will critically analyze the literature surrounding PBC in males.

View Article: PubMed Central - PubMed

Affiliation: Institute of Liver Studies, King's College London School of Medicine, Denmark Hill Campus, London SE59PJ, UK.

ABSTRACT
Primary biliary cirrhosis (PBC) is a cholestatic liver disease of autoimmune origin, characterised by the destruction of small intrahepatic bile ducts. The disease has an unpredictable clinical course but may progress to fibrosis and cirrhosis. The diagnostic hallmark of PBC is the presence of disease-specific antimitochondrial antibodies (AMA), which are pathognomonic for the development of PBC. The disease overwhelmingly affects females, with some cases of male PBC being reported. The reasons underlying the low incidence of males with PBC are largely unknown. Epidemiological studies estimate that approximately 7-11% of PBC patients are males. There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma. Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive. This paper will critically analyze the literature surrounding PBC in males.

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Histological staging in a representative case of a patient with primary biliary. Stage 1 reveals duct-centred inflammation showing chronic nonsuppurative destructive cholangitis (black arrows). A tiny granuloma is also seen (grey arrow). Stage 2 shows portal enlargement (arrows) with bile ductular reaction and inflammatory cell infiltration. Stage 3 is characterized by fibrous scaring bridging portal tracts with occasional foci of bile duct loss (no bile duct identified around an artery indicated by arrow). Stage 4 shows cirrhotic transformation.
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fig1: Histological staging in a representative case of a patient with primary biliary. Stage 1 reveals duct-centred inflammation showing chronic nonsuppurative destructive cholangitis (black arrows). A tiny granuloma is also seen (grey arrow). Stage 2 shows portal enlargement (arrows) with bile ductular reaction and inflammatory cell infiltration. Stage 3 is characterized by fibrous scaring bridging portal tracts with occasional foci of bile duct loss (no bile duct identified around an artery indicated by arrow). Stage 4 shows cirrhotic transformation.

Mentions: The diagnosis of PBC is based on three widely accepted criteria: biochemical signs of cholestasis, seropositivity for disease-specific autoantibodies, and disease-characteristic histological features (Figure 1) [1–3]. PBC-characteristic histological features include destruction of biliary epithelial cells (BECs) and loss of small bile ducts with portal inflammatory cell infiltration and granuloma formation on occasion [1–3]. Cholestatic markers include increased levels of alkaline phosphatase (ALP) and gamma-glutamyl transferase (γGT). IgM may be raised, and the most prominent immunological feature of the disease is the presence of high-titre antibodies against mitochondrial (AMA) and nuclear (ANA) antigens [1–3, 15–24]. AMAs do not appear to have clinical significance; however, disease-specific ANA can identify a subgroup of PBC patients with more advanced disease [15, 16, 24–36]. The presence of ANA at diagnosis seems to be able to identify individuals who will develop advanced disease faster than those seronegative for these autoantibodies [37]. It should be noted that patients may initially be found to be seronegative for AMA or disease-specific ANA with conventional techniques such as that of indirect immunofluorescence (IFL) [20, 38–40]. More sensitive tests using as antigenic source hybrids containing the major mitochondrial antigens have led to the appreciation that “true” AMA-seronegative cases may exist but are fewer than those considered in the past when conventional AMA testing was based on IFL and enzyme immunoassays based on the M2 antigen [20, 38–40].


Sex differences associated with primary biliary cirrhosis.

Smyk DS, Rigopoulou EI, Pares A, Billinis C, Burroughs AK, Muratori L, Invernizzi P, Bogdanos DP - Clin. Dev. Immunol. (2012)

Histological staging in a representative case of a patient with primary biliary. Stage 1 reveals duct-centred inflammation showing chronic nonsuppurative destructive cholangitis (black arrows). A tiny granuloma is also seen (grey arrow). Stage 2 shows portal enlargement (arrows) with bile ductular reaction and inflammatory cell infiltration. Stage 3 is characterized by fibrous scaring bridging portal tracts with occasional foci of bile duct loss (no bile duct identified around an artery indicated by arrow). Stage 4 shows cirrhotic transformation.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3369468&req=5

fig1: Histological staging in a representative case of a patient with primary biliary. Stage 1 reveals duct-centred inflammation showing chronic nonsuppurative destructive cholangitis (black arrows). A tiny granuloma is also seen (grey arrow). Stage 2 shows portal enlargement (arrows) with bile ductular reaction and inflammatory cell infiltration. Stage 3 is characterized by fibrous scaring bridging portal tracts with occasional foci of bile duct loss (no bile duct identified around an artery indicated by arrow). Stage 4 shows cirrhotic transformation.
Mentions: The diagnosis of PBC is based on three widely accepted criteria: biochemical signs of cholestasis, seropositivity for disease-specific autoantibodies, and disease-characteristic histological features (Figure 1) [1–3]. PBC-characteristic histological features include destruction of biliary epithelial cells (BECs) and loss of small bile ducts with portal inflammatory cell infiltration and granuloma formation on occasion [1–3]. Cholestatic markers include increased levels of alkaline phosphatase (ALP) and gamma-glutamyl transferase (γGT). IgM may be raised, and the most prominent immunological feature of the disease is the presence of high-titre antibodies against mitochondrial (AMA) and nuclear (ANA) antigens [1–3, 15–24]. AMAs do not appear to have clinical significance; however, disease-specific ANA can identify a subgroup of PBC patients with more advanced disease [15, 16, 24–36]. The presence of ANA at diagnosis seems to be able to identify individuals who will develop advanced disease faster than those seronegative for these autoantibodies [37]. It should be noted that patients may initially be found to be seronegative for AMA or disease-specific ANA with conventional techniques such as that of indirect immunofluorescence (IFL) [20, 38–40]. More sensitive tests using as antigenic source hybrids containing the major mitochondrial antigens have led to the appreciation that “true” AMA-seronegative cases may exist but are fewer than those considered in the past when conventional AMA testing was based on IFL and enzyme immunoassays based on the M2 antigen [20, 38–40].

Bottom Line: There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma.Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive.This paper will critically analyze the literature surrounding PBC in males.

View Article: PubMed Central - PubMed

Affiliation: Institute of Liver Studies, King's College London School of Medicine, Denmark Hill Campus, London SE59PJ, UK.

ABSTRACT
Primary biliary cirrhosis (PBC) is a cholestatic liver disease of autoimmune origin, characterised by the destruction of small intrahepatic bile ducts. The disease has an unpredictable clinical course but may progress to fibrosis and cirrhosis. The diagnostic hallmark of PBC is the presence of disease-specific antimitochondrial antibodies (AMA), which are pathognomonic for the development of PBC. The disease overwhelmingly affects females, with some cases of male PBC being reported. The reasons underlying the low incidence of males with PBC are largely unknown. Epidemiological studies estimate that approximately 7-11% of PBC patients are males. There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma. Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive. This paper will critically analyze the literature surrounding PBC in males.

Show MeSH
Related in: MedlinePlus