Fatigue and pruritus at onset identify a more aggressive subset of primary biliary cirrhosis.
OBJECTIVE
In recent years, primary biliary cirrhosis is mostly diagnosed in patients who are asymptomatic; however, a proportion of cases still present with typical complaints such as fatigue and/or pruritus. We compared biochemical, histological and immunological features of patients with or without fatigue and/or pruritus at onset to see whether the different clinical presentation may eventually impact on disease progression.


METHODS
We analysed the Bologna cohort of 216 patients with primary biliary cirrhosis referred to our Centre between 1997 and 2007, according to symptomatic (fatigue and/or pruritus) or asymptomatic presentation. Clinical, biochemical, histological and immunological feature at diagnosis, response to ursodeoxycholic acid and progression of the disorder were compared after a mean follow-up of 81 ± 75 months.


RESULTS
At diagnosis, symptomatic patients were significantly more often women (98.6% vs. 87.2%, P = 0.004), younger (mean age 49 ± 12 vs. 55 ± 12 years, P = 0.003) and with more pronounced biochemical activity, as indicated by higher alkaline phosphatase (mean 2.93 ± 2 vs. 2.12, P = 0.002) and aminotransferase (mean 1.92 ± 1 vs. 1.47 ± 1.27, P = 0.014) levels, whereas histological stage and autoantibody profile were similar. Symptomatic patients were less likely to respond to ursodeoxycholic acid therapy (63% vs. 81%, P = 0.006) and developed more often cirrhosis and its complications (31% vs. 13%, P = 0.004).


CONCLUSIONS
Fatigue and/or pruritus at onset identify a subset of patients with primary biliary cirrhosis who preferentially are women, younger, with a particularly active disease, less responsive to ursodeoxycholic acid treatment, and more inclined to evolve to cirrhosis and its complications.
Department of Medical and Surgical Sciences, Alma Mater Studiorum - University of Bologna, Azienda Ospedaliera Universitaria Sant'Orsola-Malpighi, Bologna, Italy.
Chiara
C
Quarneti
Paolo
P
Muratori
Claudine
C
Lalanne
Angela
A
Fabbri
Rita
R
Menichella
Alessandro
A
Granito
Chiara
C
Masi
Marco
M
Lenzi
Fabio
F
Cassani
Georgios
G
Pappas
Luigi
L
Muratori
D000328:Adult; D000469:Alkaline Phosphatase / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D015153:Blotting, Western; D015331:Cohort Studies; D018450:Disease Progression; D004797:Enzyme-Linked Immunosorbent Assay; D005221:Fatigue / Q000473:pathology*; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy; D053208:Kaplan-Meier Estimate; D008105:Liver Cirrhosis, Biliary / Q000145:classification* / Q000188:drug therapy* / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011537:Pruritus / Q000473:pathology*; D000637:Transaminases / Q000097:blood; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D001323:Autoantibodies; D014580:Ursodeoxycholic Acid; D000637:Transaminases; D000469:Alkaline Phosphatase
antimitochondrial antibodies; biliary; cirrhosis; symptomatic; therapy