Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community.
OBJECTIVE
The epidemiology of primary sclerosing cholangitis (PSC) in the United States is unknown. We report the incidence, clinical spectrum, and outcomes of PSC in Olmsted County, Minnesota.


METHODS
Using the Rochester Epidemiology Project, a medical records linkage system in Olmsted County, Minnesota, we identified county residents with PSC, and the diagnosis was confirmed according to clinical, biochemical, radiographic, and histologic criteria.


RESULTS
Twenty-two patients met diagnostic criteria for PSC in 1976-2000. The age-adjusted (to 2000 U.S. whites) incidence of PSC in men was 1.25 per 100,000 person-years (95% CI, 0.70 to 2.06) compared with 0.54 per 100,000 person-years (95% CI, 0.22 to 1.12) in women. The prevalence of PSC in 2000 was 20.9 per 100,000 men (95% CI, 9.5 to 32.4) and only 6.3 per 100,000 women (95% CI, 0.1 to 12.5). Seventy-three percent of cases had inflammatory bowel disease, the majority with ulcerative colitis. Survival among PSC patients was significantly less than expected for the Minnesota white population of similar age and gender (P < 0.001).


CONCLUSIONS
These data represent the first population-based estimates of the incidence and prevalence of PSC in the United States. The incidence and prevalence of PSC were approximately one third of those previously described for primary biliary cirrhosis in the same population. Our data suggest that the prevalence of PSC in the United States, with its attendant medical burdens, is significantly greater than previously estimated.
Bambha
Kiran
K
Division of Gastroenterology and Hepatology, Mayo Medical School, Clinic and Foundation, 200 First Street SW, Rochester, MN 55905, USA.
Kim
W Ray
WR
Talwalkar
Jayant
J
Torgerson
Heidi
H
Benson
Joanne T
JT
Therneau
Terry M
TM
Loftus
Edward V
EV
Yawn
Barbara P
BP
Dickson
E Rolland
ER
Melton
L Joseph
LJ
D000328:Adult; D000368:Aged; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000453:epidemiology* / Q000503:physiopathology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D015212:Inflammatory Bowel Diseases / Q000150:complications; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009026:Mortality; D015995:Prevalence; D017678:Sex Distribution; D014481:United States / Q000453:epidemiology