QJM Advance Access originally published online on April 14, 2008
QJM 2008 101(6):505-506; doi:10.1093/qjmed/hcn016
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Clinical and serological profile of primary biliary cirrhosis in young and elderly patients
Sir,Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that affects mainly middle age women1,2 with a slowly progression to cirrhosis. Little is known about the clinical expression of PBC in younger and elderly patients. We analysed the clinical profile of patients at presentation on the basis of the age at diagnosis.
One hundred and ninety-eight patients who satisfied the established criteria for a diagnosis of PBC3 were included in this study and subdivided in three groups: group A (including patients with an age <45 years—44 cases), group B (with an age between 45 and 65 years—105 cases) and group C (with an age >65 years—49 cases).
Clinical history, physical examination, routine biochemical and immunological tests were performed in each patient.
Histology was available in 153 (77%) patients, and the stage (I–IV) has been attributed on the basis of the presence of the most advanced lesion in the biopsy specimen.4
As expected, group B was the most represented category (105 of 198, 53%) with respect to group A 44 (22%) and C 49 (25%) patients (P < 0.0001 and P = 0.0001, respectively). Group A presented a more ‘cytolitic pattern’ expressed by significantly higher serum levels of transaminases than group B and C (P = 0.0001 and 0.0024, respectively); furthermore, group A and B were significant more symptomatic at diagnosis than older patients (59% and 47% versus 26%; P = 0.001 and P = 0.02 respectively).
The most represented symptoms at presentation was represented by pruritus and fatigue; in particular pruritus affected group A (21%) more than group B (11%) and group C (13%), even if the statistical significance was not reached.
In elderly, the frequency of male gender was significantly higher than in middle and youger age (40% versus 8% and 9%, respectively, P = 0.002 and 0.003);5 Serologically, all three studied group presented the same rate of frequency of the PBC-specific reactivities (AMA, MND, Rim-like/Membranous and anti-centromere antibodies- Table 1).
|
Under histological profile, older patients (group C) were significantly less affected by initial histological stage 19% than younger (63%, P = 0.003) and middle age patients (70%, P < 0.001).
Younger patients at presentation displayed a higher biochemical activity and more symptomatic disease; this could be cause of early diagnosis in this fashion of patients with respect to the other ones. The progression of the disease seems to be independent by the clinical expression given the low degree of initial histological stage in elderly patients where the frequency of asymptomaticity is high.
On the basis of our analysis, we conclude that possibly two types of phenotipic expression of PBC exist; the first is the classical asymptomatic onset in the middle-late age with a mild biochemical activity, the other one is a symptomatic onset in young age with a high biochemical (cytolitic) activity.
Department of Internal Medicine, Cardioangiology
Hepatology, Alma Mater Studiorum
University of Bologna, S. Orsola-Malpighi Hospital
Via Massarenti 9, 40138, 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][Web of Science][Medline]
4. Scheuer PJ. Primary biliary cirrhosis. Proct R Soc Med (1967) 60:1257.
5. Muratori P, Granito A, Pappas G, Muratori L, Quarneti C, De Molo C, et al. Clinical and serological profile of primary biliary cirrhosis in men. Q J Med (2007) 100:534–5.[Web of Science]
CiteULike 
Connotea 
Del.icio.us What's this?
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||