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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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Clinical and Laboratory differences between women and men with PBC. The figure illustrates the significant differences between the Italian cohorts of men and women with PBC analysed by Muratori et al. [52] Only the parameters that reached statistically significant difference are given. More details are provided within the text; UNL, upper normal level; histo, histological; CEN, centromere; abs, antibodies.
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fig2: Clinical and Laboratory differences between women and men with PBC. The figure illustrates the significant differences between the Italian cohorts of men and women with PBC analysed by Muratori et al. [52] Only the parameters that reached statistically significant difference are given. More details are provided within the text; UNL, upper normal level; histo, histological; CEN, centromere; abs, antibodies.

Mentions: Another study, conducted by a specialist liver unit at King's College Hospital, compared the clinical and biochemical profiles of males and females with PBC, in addition to long-term outcome [128]. Thirty-nine (39) men and 191 women with PBC were enrolled, with the age of diagnosis and disease severity being similar in both groups. Pruritus was found to be less common in males than females (45% versus 68%), and it was suggested that female sex hormones may be linked with pruritus, as there was an increased frequency of women reporting pruritus beginning with the administration of oral contraceptives, as well as during pregnancy [128]. Much like the earlier study [127], the group at King's College Hospital found that gastrointestinal bleeding was more common among males patients (23%) than female patients (15%), although this was not indicated to be statistically significant [128]. In fact, the only statistically significant finding in regards to clinical signs was that females demonstrated skin pigmentation more often than males (55% versus 35%) [128]. Concomitant autoimmune diseases were also examined between the two groups. Sicca symptoms were present in 33% of females and 15% males, scleroderma in 13% of females and 8% of males, and Raynauds in 13% females and 3% of males [128]. These findings suggest that females were more likely to suffer concomitant autoimmune disease than males. Interestingly, that study was the first to report an increased frequency of type 2 diabetes in male PBC patients [128]. As well, men with PBC had a higher frequency of hepatoma, which has also been found in other studies [128–130]. No significant differences were observed in regards to AMA, biochemistry, histology, or survival [128]. An Italian study [52] compared clinical and serological data of 30 consecutive male and 165 female PBC patients (Figure 2). Histology was available in 83% of the males and 79% of females. Clinically, there was a significant difference in age between the two groups, with males presenting at a median age of 68.5 years compared to 54.5 years in females [52]. Scleral jaundice was more common among males (13%) than females (11%), which was the only statistically significant clinical difference among the two groups (Figure 2). However, it should be noted that 64% of males and 51.5% of females were asymptomatic at the time of diagnosis, although this was not statistically significant [52]. Biochemically, males had higher levels of ALT and γGT [52]. Analysis of biopsy specimens revealed that stage I was present in 35% of females compared to 12% of males, although 36% and 28% of males were in stages III and IV, respectively, compared to 19% of females in both stages [52]. However, this was not found to be statistically significant. Immunological profiles regarding AMA and ANA were not different between the two groups, although a higher frequency of anti-centromere activity was noted in females (21.4%) than in males (3%) [52]. That study concluded that more advanced disease in males was most likely due to delayed diagnosis in this group, in which PBC is not initially suspected [52].


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)

Clinical and Laboratory differences between women and men with PBC. The figure illustrates the significant differences between the Italian cohorts of men and women with PBC analysed by Muratori et al. [52] Only the parameters that reached statistically significant difference are given. More details are provided within the text; UNL, upper normal level; histo, histological; CEN, centromere; abs, antibodies.
© Copyright Policy
Related In: Results  -  Collection

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

fig2: Clinical and Laboratory differences between women and men with PBC. The figure illustrates the significant differences between the Italian cohorts of men and women with PBC analysed by Muratori et al. [52] Only the parameters that reached statistically significant difference are given. More details are provided within the text; UNL, upper normal level; histo, histological; CEN, centromere; abs, antibodies.
Mentions: Another study, conducted by a specialist liver unit at King's College Hospital, compared the clinical and biochemical profiles of males and females with PBC, in addition to long-term outcome [128]. Thirty-nine (39) men and 191 women with PBC were enrolled, with the age of diagnosis and disease severity being similar in both groups. Pruritus was found to be less common in males than females (45% versus 68%), and it was suggested that female sex hormones may be linked with pruritus, as there was an increased frequency of women reporting pruritus beginning with the administration of oral contraceptives, as well as during pregnancy [128]. Much like the earlier study [127], the group at King's College Hospital found that gastrointestinal bleeding was more common among males patients (23%) than female patients (15%), although this was not indicated to be statistically significant [128]. In fact, the only statistically significant finding in regards to clinical signs was that females demonstrated skin pigmentation more often than males (55% versus 35%) [128]. Concomitant autoimmune diseases were also examined between the two groups. Sicca symptoms were present in 33% of females and 15% males, scleroderma in 13% of females and 8% of males, and Raynauds in 13% females and 3% of males [128]. These findings suggest that females were more likely to suffer concomitant autoimmune disease than males. Interestingly, that study was the first to report an increased frequency of type 2 diabetes in male PBC patients [128]. As well, men with PBC had a higher frequency of hepatoma, which has also been found in other studies [128–130]. No significant differences were observed in regards to AMA, biochemistry, histology, or survival [128]. An Italian study [52] compared clinical and serological data of 30 consecutive male and 165 female PBC patients (Figure 2). Histology was available in 83% of the males and 79% of females. Clinically, there was a significant difference in age between the two groups, with males presenting at a median age of 68.5 years compared to 54.5 years in females [52]. Scleral jaundice was more common among males (13%) than females (11%), which was the only statistically significant clinical difference among the two groups (Figure 2). However, it should be noted that 64% of males and 51.5% of females were asymptomatic at the time of diagnosis, although this was not statistically significant [52]. Biochemically, males had higher levels of ALT and γGT [52]. Analysis of biopsy specimens revealed that stage I was present in 35% of females compared to 12% of males, although 36% and 28% of males were in stages III and IV, respectively, compared to 19% of females in both stages [52]. However, this was not found to be statistically significant. Immunological profiles regarding AMA and ANA were not different between the two groups, although a higher frequency of anti-centromere activity was noted in females (21.4%) than in males (3%) [52]. That study concluded that more advanced disease in males was most likely due to delayed diagnosis in this group, in which PBC is not initially suspected [52].

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