QJM Advance Access originally published online on July 3, 2007
QJM 2007 100(8):534-535; doi:10.1093/qjmed/hcm059
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Clinical and serological profile of primary biliary cirrhosis in men
Sir,Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by a high ratio of women to men (9:1).1,2 Little is known about the clinical expression of PBC in men, since the rarity of this disease in males makes it difficult to enrol sufficient numbers in a study. We analysed the clinical profile of male patients with PBC at presentation, and compared them with a control group of their female counterparts.
Thirty consecutive men and 165 women who satisfied the established criteria for the diagnosis of PBC were included.3 Clinical history, physical examination, and routine biochemical and immunological tests were done in each patient. Histology was available in 25 men (83%) and 130 women (79%), graded stage (I–IV) on the basis of the most advanced lesion in the biopsy specimen.4
Men were typically older than women (median 68.5 [range 34–81] vs. 54.5 [range 25–96] years, p = 0.002) and more frequently had scleral jaundice (13% vs. 1%, p = 0.002); they also had higher ALT (1.7 UNL [upper normal limit] vs. 1 UNL, p = 0.01) and higher 
-GT serum levels (4.9 UNL vs. 2.9 UNL, p = 0.0035) (Table 1). Men had a lower frequency of stage I histology compared to women (12% vs. 35%, p = 0.03). There were no differences between men and women with respect to anti-mitochondrial, multiple nuclear dot or rim-like/membranous serological patterns, but there was a significantly lower frequency of anti-centromere reactivity in men vs. women (3% vs. 21.4%, p = 0.02).
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Because of the significant difference in age at diagnosis between men and women, we also compared the male group with 60 age-matched female controls; all the clinical, biochemical and histological differences, except
-GT serum levels, then disappeared. More than half of our patients had a diagnosis of PBC when they were still asymptomatic and only a biochemical cholestasis was present. At presentation, men with PBC were older, with higher hepatocytolytic and cholestatic activity than women, and were less frequently in stage 1 at liver biopsy; taken together, these findings might suggest that in males, PBC is a more aggressive disease than in females. In a recent paper by Nakamura et al., male gender was considered an independent risk factor for disease progression.5
However, male gender does not appear to be associated with any evidence of disease progression. It is possible that the older age of males at diagnosis merely reflects a delayed diagnosis, and thus more advanced disease. In other words, we suspect that in male patients the demonstration of intrahepatic cholestasis does not immediately suggest an autoimmune event, as in the case of PBC, and therefore does not prompt a search for PBC-specific AMA and ANA. This hypothesis is supported by our observations on age-matched controls, where all differences except -GT disappeared.
In over 50% of patients, irrespective of gender, the diagnosis of PBC is performed when the disease is still asymptomatic. The more advanced clinical picture at presentation in men is probably the result of a delay in diagnosis. Although rare, a diagnosis of PBC should be considered in male patients with intrahepatic cholestasis.
Departments of Internal Medicine,
Cardioangiology and Hepatology,
Alma Mater Studiorum University of Bologna,
S.Orsola-Malpighi Hospital,
Bologna Italy
email: paolo.muratori3{at}unibo.it
References
1. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med (2006) 354:313.
2. Pares A, Rodes J. Natural history of primary biliary cirrhosis. Clin Liver Dis (2003) 7:779–94.[CrossRef][Medline]
3. Heathcote EJ. Management of Primary Biliary Cirrhosis. The American Association for the study of liver disease. Practice guidelines. Hepatology (2000) 31:1005–13.[CrossRef][ISI][Medline]
4. Scheuer PJ. Primary Biliary Cirrhosis. Proc R Soc Med (1967) 60:1257–60.[ISI][Medline]
5. Nakamura M, Kondo H, Mori T, et al. Anti-gp210 and anti-centromere antibodies are different risk factors for the progression of primary biliary cirrhosis. Hepatology (2007) 45:118–27.[CrossRef][ISI][Medline]
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