Background & Aims: Seventy percent of patients with primary sclerosing
cholangitis (PSC) have concomitant ulcerative colitis. Smoking and pr
evious appendectomy may protect against ulcerative colitis. The aim of
this study was to examine these factors in patients with PSC. Methods
: Fifty-nine patients with PSC, 130 patients with ulcerative colitis a
nd normal liver biochemistry, and 197 control subjects were interviewe
d about smoking behavior and history of appendectomy. Results: There w
ere less current smokers in the PSC and ulcerative colitis groups than
in the control group (19%, 12%, and 38%, respectively). The resulting
odds ratio for current smoking was 0.37 (95% confidence interval, 0.1
8-0.76) in the PSC group and 0.23 (95% confidence interval, 0.13-0.41)
in the ulcerative colitis group. Percentage of persons who ever smoke
d was also significantly less in the PSC group (41% vs. 56% in the con
trol group). Frequency of previous appendectomy in the PSC and ulcerat
ive colitis groups was not significantly different from that of contro
ls (19%, 9%, and 14%, respectively). Conclusions: Smoking but not prev
ious appendectomy is associated with decreased risk of PSC.