Secular trends in incidence and 30-day case fatality of acute pancreatitis in North Jutland County,Denmark: a register-based study from 1981-2000

BACKGROUND: The incidence rate of acute pancreatitis has been reported as having increased during recent decades in Western countries. Reported mortality lies around 10% and has improved during the past 20 years. The incidence rate and 30-day case fatality rate of acute pancreatitis in North Jutland County, Denmark were examined for the period 1981 to 2000. METHODS: Data were collected from the Hospital Discharge Registry of North Jutland County for the period 1981-2000. Sex- and age-standardized incidence rates and 30-day case fatality rate of a first attack of acute pancreatitis were calculated. Data on endoscopic procedures were assessed for the period 1992 to 2000 and on certain drugs for 1991 to 1999. RESULTS: The incidence rate of acute pancreatitis in women increased from 17.1 per 100,000 person-years in 1981 (95% confidence interval (CI), 12.6-23.2) to 37.8 per 100,000 person-years in 2000 (95% CI, 31.0-46.1). The corresponding increase in men was from 18 per 100,000 person-years in 1981 (95% CI, 13.3-24.2) to 27.1 per 100,000 person-years in 2000 (95% CI, 21.5-34.3). The incidence rate of acute pancreatitis increased with age in both sexes. The overall 30-day case fatality rate was 7.5% (95% CI, 6.5-8.7) increasing with age, adjusted odds ratio (OR) = 6.4 (95% CI, 3.5-11.6) and decreased with time, adjusted OR = 0.7 (95% CI, 0.4-1.0). CONCLUSION: The incidence of acute pancreatitis has increased, and in women surpassed that in men in 1999 and 2000. Short-term prognosis has improved.     

Increase in incidence and prevalence of inflammatory bowel disease in northern Denmark: a population-based study, 1978-2002

OBJECTIVES: Although incidence rates of inflammatory bowel disease have been reported worldwide, few long-term population-based studies with current time-trend analyses exist. We therefore examined time trends in the incidence rate of inflammatory bowel disease in a 25-year study period, and estimated the prevalence in 2002. All patients diagnosed between 1978 and 2002 were included as incident cases (n=2,326) and all patients living in North Jutland County on 31 December 2002 were used to estimate prevalent cases (n=2,205). METHODS: Medical records of all patients diagnosed with ulcerative colitis and Crohn's disease in the North Jutland County Hospital Discharge Registry were reviewed to examine if the diagnostic criteria were fulfilled. Age-specific and gender-specific standardized incidence rates were calculated. RESULTS: For ulcerative colitis, incidence rates in women increased from 8.3 (95% confidence interval (CI): 6.7-9.9) in 1978-1982 to 17.0 (95% CI: 14.7-19.3) per 100,000 person-years in 1998-2002. The corresponding figures for men were 7.7 (95% CI: 6.1-9.3) and 16.7 (95% CI: 14.4-18.8) per 100,000 person-years. For Crohn's disease, the incidence rates in women increased from 4.1 (95% CI: 3.0-5.2) in 1978-1982 to 10.7 (95% CI: 8.8-12.5) per 100,000 person-years in 1998-2002. The corresponding figures for men were 3.2 (95% CI: 2.1-4.2) and 8.5 (95% CI: 6.9-10.2) per 100,000 person-years. The prevalence of ulcerative colitis and Crohn's disease was 294 and 151 per 100,000 inhabitants, respectively. CONCLUSIONS: A marked and parallel increase was seen in both ulcerative colitis and Crohn's disease in both genders during the last 25 years, with a corresponding high prevalence of both diseases.     

Risk and case fatality rate of meningitis in patients with liver cirrhosis

Bacterial infections are major causes of morbidity and death in patients with liver cirrhosis. The risk of bacterial meningitis in these patients is unknown, however. In this study on a nation-wide cohort of 22,743 patients with liver cirrhosis in Denmark an incidence rate of bacterial meningitis of 54.4 per 100,000 was found [95% confidence interval (CI) 40.3-71.9]. The highest incidence rate was found in patients with alcoholic cirrhosis, 65.3 per 100,000 person-years (95% CI 46.2-89.6), compared with 34.6 per 100,000 person-years (95% CI 17.3-61.9) in patients with non-alcoholic cirrhosis. The 30-d case fatality rate was 53.1% (95% CI 38.3-67.5), and high age and alcoholic cirrhosis were associated with the highest case fatality rates. The main bacterial pathogens were pneumococci and unspecified bacteria. These findings suggest that patients with liver cirrhosis are at increased risk of bacterial meningitis with a poor prognosis.     

Incidence and mortality of acute pancreatitis between 1985 and 1995

BACKGROUND: The incidence of acute pancreatitis seems to have increased in Western countries. It has been suggested that this increase can be explained by improved diagnostic procedures. We performed a nationwide study to assess the annual sex- and age-specific incidence and mortality rates of acute pancreatitis in the Netherlands between 1985 and 1995, a period in which diagnostic procedures did not change considerably. METHODS: We conducted a population-based retrospective follow-up study in which we used automated hospital discharge data accumulated by Prismant Health Care Information. All patients admitted with acute pancreatitis (ICD-9CM, 577.0) in the Netherlands were identified. We accounted for referrals to other hospitals to avoid double counting and for miscoding of chronic pancreatitis as acute pancreatitis. The annual population size was retrieved from the Netherlands Central Statistics Office. RESULTS: The observed incidence of acute pancreatitis increased from 12.4/100,000 person-years (95% confidence interval (CI), 11.8-12.9) in 1985 to 15.9/100,000 person-years (95% CI, 15.3-16.5) in 1995. The annual mortality rate of acute pancreatitis remained fairly stable at 1.5/100,000 person-years. The incidence and mortality rate of acute pancreatitis increased considerably with age. The case-fatality proportion of first admissions for acute pancreatitis decreased from 14.3% to 10.7%. The case-fatality for relapses remained stable at 3.2%. CONCLUSIONS: In this retrospective study the observed incidence of acute pancreatitis increased by 28% between 1985 and 1995. Due to a decrease in the case-fatality proportion, the mortality remained stable during this period.     

Trends and forecasts of hospital admissions for acute and chronic pancreatitis in the Netherlands

BACKGROUND: The incidence and prevalence of acute and chronic pancreatitis have increased in Western countries. It is likely, the number of hospital admissions has increased correspondingly. AIMS: To analyze the trends in hospital admissions in the Netherlands for acute and chronic pancreatitis from 1992 to 2004 and to forecast the number of admissions up to 2010. METHODS: Analysis of hospital admissions for acute and chronic pancreatitis accumulated in a nationwide database. Curve fitting regression models were used to explore future trends. RESULTS: The number of acute pancreatitis admissions rose in 1992-2004 from 1,785 to 3,120 (74.8% increase). The overall 'annual number' of acute pancreatitis admissions increased from 11.8 to 19.2 per 100,000 person-years. The linear regression model predicted 3,205 [95% confidence intervals (CI), 3,111-3,299] and 3,537 (95% CI, 3,429-3,645) admissions for 2007 and 2010, respectively, a further increase of at least 9.9% in 2010 compared with 2004. In the 12-year time period, chronic pancreatitis admissions showed an increase of 75.4% (from 790 to 1,386). The overall 'annual number' of chronic pancreatitis admissions increased from 5.2 to 8.5 per 100,000 person-years. The cubic regression model predicted 1868 (95% CI, 1,619-2,117) and 3,173 (95% CI, 2,456-3,890) admissions for 2007 and 2010, respectively, an additional increase of 77.2% in 2010 compared with 2004. CONCLUSION: Hospital admissions for acute and chronic pancreatitis have increased substantially from 1992-2004. This trend will most likely continue for the near future and the burden and costs to the Dutch health care system will increase accordingly.     

Incidence and Mortality of Acute Pancreatitis between 1985 and 1995

Background: The incidence of acute pancreatitis seems to have increased in Western countries. It has been suggested that this increase can be explained by improved diagnostic procedures. We performed a nationwide study to assess the annual sex- and age-specific incidence and mortality rates of acute pancreatitis in the Netherlands between 1985 and 1995, a period in which diagnostic procedures did not change considerably. Methods: We conducted a population-based retrospective follow-up study in which we used automated hospital discharge data accumulated by Prismant Health Care Information. All patients admitted with acute pancreatitis (ICD-9CM, 577.0) in the Netherlands were identified. We accounted for referrals to other hospitals to avoid double counting and for miscoding of chronic pancreatitis as acute pancreatitis. The annual population size was retrieved from the Netherlands Central Statistics Office. Results: The observed incidence of acute pancreatitis increased from 12.4/100,000 person-years (95% confidence interval (CI), 11.8-12.9) in 1985 to 15.9/100,000 person-years (95% CI, 15.3-16.5) in 1995. The annual mortality rate of acute pancreatitis remained fairly stable at 1.5/100,000 person-years. The incidence and mortality rate of acute pancreatitis increased considerably with age. The case-fatality proportion of first admissions for acute pancreatitis decreased from 14.3% to 10.7%. The case-fatality for relapses remained stable at 3.2%. Conclusions: In this retrospective study the observed incidence of acute pancreatitis increased by 28% between 1985 and 1995. Due to a decrease in the case-fatality proportion, the mortality remained stable during this period.     

Hypertriglyceridemia is often under recognized as an aetiologic risk factor for acute pancreatitis: A population-based cohort study

BackgroundSevere hypertriglyceridemia (HTG) is a well-known risk factor for acute pancreatitis, but updated population-based estimates on incidence of HTG-associated pancreatitis are lacking.MethodsWe identified all individuals with severe HTG (triglyceride level >10 mmol/L [886 mg/dL]) in a population-based sample from 2008 to 2019 and linked these with Danish nationwide health-registers to identify patients with acute pancreatitis. Pancreatitis cases were subsequently confirmed by a detailed medical chart review. Crude and standardized incidence rates were estimated and studied in relation to age, gender and time-period. In addition, aetiological classification designated during index hospitalization, severity and follow-up of individuals with HTG-associated pancreatitis were studied.ResultsAmong 2146 individuals with severe HTG during the observation period, 75 were diagnosed with acute pancreatitis (3.5%). The mean incidence rate of HTG-associated pancreatitis was 1.4 (95% CI, 1.1–1.7) per 100,000 person years for the total population, for women it was 0.7 (95% CI, 0.5–1.1) and for men 2.0 (95% CI, 1.5–2.6) per 100,000 person-years. The mean incidence rate increased from 0.7 to 1.7 per 100,000 person-years from 2008 to 2019 (p_trend = 0.01). The highest incidence rate of HTG-associated pancreatitis was observed for men in the age group 50–59 years. An elevated triglyceride level was recognized as aetiological risk factor in 35% of patients during index hospitalization.ConclusionsOnly a fraction of patients with severe HTG are hospitalized for acute pancreatitis, but the incidence is increasing. In more than half of patients elevated triglycerides is not recognized as a risk factor for acute pancreatitis during index hospitalization.     

Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community

BACKGROUND & AIMS: 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.     

Epidemiology of sporadic inclusion body myositis and polymyositis in Olmsted County, Minnesota

OBJECTIVE: To determine the incidence and prevalence of sporadic inclusion body myositis (sIBM) and polymyositis (PM) in a population-based study. METHODS: Charts of patients with myositis in Olmsted County, Minnesota, USA, from 1981 to 2000 were reviewed. RESULTS: For sIBM, the age- and sex-adjusted incidence rates per 100,000 were 0.79 (95% confidence interval = 0.24-1.35), and for PM, 0.41 (95% CI 0.08-0.73). The age- and sex-adjusted prevalence rates per 100,000 were 7.06 (95% CI 0.87-13.24) for sIBM and 3.45 (95% CI 0.00-7.35) for PM. CONCLUSION: The incidence and prevalence rates for sIBM are higher than previously reported.     

Epidemiology of central nervous system infections in Olmsted County, Minnesota, 1950-1981

We identified all diagnosed cases of infections of the central nervous system (CNS), excluding poliomyelitis, in the population of Olmsted County, Minnesota, from 1950 to 1981 and described incidence, time trends, etiologic agents, and mortality for these infections. The adjusted incidence rate for bacterial meningitis was 8.6/100,000 person-years (with a case fatality ratio of 10%) and was highest in children less than five years of age; in this age-group, rates more than doubled from 1950 to 1981. The adjusted incidence rate of brain abscess was 1.1, with a case fatality ratio of 37%. The adjusted incidence rate of aseptic meningitis was 10.9/100,000 person-years. Age-specific rates were highest in children less than one year of age and in men, and increased during the study period. The adjusted incidence rate of viral encephalitis was 7.4, with a case fatality ratio of 3.8%. Rates were highest in children less than 10 years of age and in men. By 10 years of age, 0.9% of the men and 0.7% of the women were affected by a CNS infection. Cumulative incidence (risk) through age 80 was 2.3% for men and 1.5% for women.     

Seasonal pattern of incidence and case fatality of acute myocardial infarction in a Japanese population (from the Takashima AMI Registry, 1988 to 2003)

We examined the seasonal variations in the incidence and case fatality of acute myocardial infarction (AMI) in a geographically defined population using 16-year AMI registration data. It remains unclear whether the incident events of AMI display any seasonal variation. Documentation of such a pattern may foster investigation for understanding the potential mechanisms responsible for these effects and may provide novel avenues for prevention of AMI. Data were obtained from the Takashima AMI Registry, which covers an entire community of approximately 55,000 in central Japan. There were 335 definite cases of AMI during 1988 to 2003 (217 men, 118 women). Of these, there were 96 fatal cases (53 men, 43 women) within 28 days of onset. Incidence rates (per 100,000 person-years) and case fatality rates with 95% confidence intervals (CI) were calculated across seasons. Poisson regression analysis was used to calculate the incidence rate, and case fatality ratios adjusted for age and gender. The AMI incidence rate was higher in winter (44.9, 95% CI 35.9 to 53.9) and spring (44.1, 95% CI 35.3 to 52.9) than the other seasons. After adjustment for age and gender, AMI risk was 1.4 (95% CI 1.03 to 1.9) times higher in winter and was 1.4 (95% CI 1.01 to 1.9) times higher in spring than summer. The 28-day AMI case fatality rate was also higher in winter (34.4%, 95% CI 24.9 to 43.9) and spring (32.3%, 95% CI 22.9 to 41.7). Age- and gender-adjusted fatality risk was 2.4 (95% CI 1.2 to 4.9) times higher in winter and 2.3 (95% CI 1.1 to 4.6) times higher in spring than summer. In conclusion, higher AMI incidence and case fatality rates were observed in winter and spring in a Japanese population.     

Secular trends in cardiovascular disease mortality, incidence, and case fatality rates in adults in the United States

PURPOSE: To assess the effects of changes in cardiovascular disease incidence and case fatality rates on secular trends in mortality in the U.S. population between 1971-1982 and 1982-1992. METHODS: Using data from the first National Health and Nutrition Examination Survey Epidemiologic Follow-up Study, two cohorts (10,869 subjects in the 1971-1982 cohort and 9774 in the 1982-1992 cohort) of participants aged 35 to 74 years were created. Baseline medical history questionnaires were administered in 1971-1975 and 1982-1984, with follow-up interviews, hospital record reviews, and death certificate searches conducted in 1982-1984, 1986, 1987, and 1992. RESULTS: Between 1971-1982 and 1982-1992, age-standardized cardiovascular disease mortality declined from 79.1 (95% confidence interval [CI]: 75.2 to 83.0) to 53.0 (95% CI: 49.5 to 56.5) per 10,000 person-years, while cardiovascular disease incidence rates decreased from 293.5 (95% CI: 284.5 to 302.4) to 225.1 (95% CI: 216.6 to 233.5) per 10,000 person-years. The 28-day case fatality rate for cardiovascular disease declined from 15.7% (95% CI: 14.5% to 16.8%) to 11.7% (95% CI: 10.3% to 13.0%). After adjustment for age, sex, and race, rates were 31% lower for cardiovascular disease mortality, 21% lower for incidence, and 28% lower for 28-day case fatality in the 1982-1992 cohort than in the 1971-1982 cohort (each P <0.001). CONCLUSION: The decrease in cardiovascular disease mortality between 1971-1982 and 1982-1992 was due to declines in both the incidence and case fatality rates in this national sample. These findings suggest that both primary and secondary prevention and treatment contributed to the decline in cardiovascular disease mortality in the United States.     

Update on the incidence and prevalence of Crohn's disease and ulcerative colitis in Olmsted County, Minnesota, 1940-2000

BACKGROUND: We previously reported that the prevalence of Crohn's disease (CD) and ulcerative colitis (UC) in Olmsted County, Minnesota, had risen significantly between 1940 and 1993. We sought to update the incidence and prevalence of these conditions in our region through 2000. METHODS: The Rochester Epidemiology Project allows population-based studies of disease in county residents. CD and UC were defined by previously used criteria. County residents newly diagnosed between 1990 and 2000 were identified as incidence cases, and persons with these conditions alive and residing in the county on January 1, 2001, were identified as prevalence cases. All rates were adjusted to 2000 US Census figures for whites. RESULTS: In 1990-2000 the adjusted annual incidence rates for UC and CD were 8.8 cases per 100,000 (95% confidence interval [CI], 7.2-10.5) and 7.9 per 100,000 (95% CI, 6.3-9.5), respectively, not significantly different from rates observed in 1970-1979. On January 1, 2001, there were 220 residents with CD, for an adjusted prevalence of 174 per 100,000 (95% CI, 151-197), and 269 residents with UC, for an adjusted prevalence of 214 per 100,000 (95% CI, 188-240). CONCLUSION: Although incidence rates of CD and UC increased after 1940, they have remained stable over the past 30 years. Since 1991 the prevalence of UC decreased by 7%, and the prevalence of CD increased about 31%. Extrapolating these figures to US Census data, there were approximately 1.1 million people with inflammatory bowel disease in the US in 2000.     

Acute pancreatitis: 31-Year trends in incidence and mortality – A Danish population-based cohort study

BackgroundObjectives: Increasing incidence rates and declining mortality rates have made acute pancreatitis a common cause of hospitalization. We aimed to examine 31-year trends in first-time hospitalization for acute pancreatitis, the subsequent short-term and long-term mortality, and the prognostic impacts of age, sex, and comorbidity.MethodsIn this nationwide Danish population-based cohort study of 47,711 incident cases, we computed the annual sex-specific age-standardized incidence rates of acute pancreatitis for 1988–2018. Among patients with incident hospitalization for acute pancreatitis, we computed sex-specific 30-day and 31–365-day mortality rates, stratified them, and performed proportional-hazards regression to estimate mortality rate ratios adjusted for sex, age, and comorbidity, measured by Charlson Comorbidity Index categories.ResultsFrom 1988 to 2018, the standardized incidence rate of acute pancreatitis per 100,000 person-years increased by 29% for men (28.8–37.0%) and by 148% for women (15.7–38.9%). Among patients with pancreatitis, the 30-day mortality declined from 10.0% in those diagnosed in 1988–1992 to 6.3% for those diagnosed in 2013–2017. The corresponding 31–365 day mortality increased from 5.5% to 6.0%. In comparing periods 1988–1992 and 2013–17, the adjusted 30-day mortality rate ratio was 0.36 (95% confidence interval: 0.32–0.41) and the adjusted 31–365 day mortality rate ratio was 0.64 (95% confidence interval: 0.56–0.74). Comorbidity was a strong predictor of mortality among patients with pancreatitis.ConclusionsOver the 31 years of observations, annual rates of acute pancreatitis more than doubled among women, converging with those among men. The comorbidity burden was a strong prognostic factor for short and long-term mortality. Treatments for acute pancreatitis should focus on existing comorbidities.     

Trends in the incidence of polycythemia vera among olmsted county,Minnesota residents, 1935–1989

To investigate the suggestion that the incidence of polycythemia vera has increased in recent decades, we ascertained secular trends in the incidence of polycythemia vera in Olmsted County, Minnesota, over the 55-year period, 1935–1989. The inpatient and outpatient medical records of all potential cases of polycythemia vera in Olmsted County residents were reviewed and the diagnostic criteria of the Polycythemia Vera Study Group were applied. We found no indication of an increase in the age- and sex-adjusted incidence of polycythemia vera, which averaged 1.9 per 100,000 person-years (95% C.I., 1.4–2.5) over the study period. Incidence rates increased with age, and age-adjusted incidence rates were greater for men (2.8 per 100,000 person-years; 95% C.I., 1.8–3.9) than for women (1.3 per 100,000 person-years; 95% C.I., 0.7–1.9), with the highest incidence rate (23.5 per 100,000 person-years) among men aged 70–79 years. Survival was reduced in this inception cohort of 50 cases, compared to that expected for individuals of like age and sex (P < 0.0001); median survival following diagnosis was 7.2 years. © 1994 Wiley-Liss, Inc.     

Population-based study of the risk and short-term prognosis for bacteremia in patients with liver cirrhosis.

We examined the risk of bacteremia in patients with liver cirrhosis (compared with the risk for all Danish citizens >20 years of age who were living in North Jutland County, Denmark), as well as the type of bacteremia and the 30-day case-fatality rate. We used the Danish National Registry of Patients to identify 1339 patients with liver cirrhosis, and we used the North Jutland County Bacteremia Database to identify episodes of bacteremia. We observed 117 cases of bacteremia in patients with liver cirrhosis (11.0 cases were expected), which yielded a standardized incidence ratio of 10.5 (95% confidence interval [CI], 8.8-12.7). Sixty-two cases of bacteremia were nosocomial infections. There were 53 cases of gram-positive bacteremia, 55 cases of gram-negative bacteremia, and 8 cases of polymicrobial bacteremia (1 case of candidemia was excluded from the analysis). The most common cause of death was bleeding from gastroesophageal varices; the second most common cause of death was infection in the respiratory system. The 30-day case-fatality rate was 0.53 (95% CI, 0.39-0.73). Patients with liver cirrhosis had an increased risk of bacteremia and a poor prognosis.     

Incidence and short-term prognosis of infective endocarditis in Denmark, 1980-1997

Infective endocarditis is a serious bacterial infection, but there are relatively few data about its occurrence and prognosis. The incidence rate, 30-d case fatality and mortality rate of infective endocarditis were estimated in this registry-based cohort study. The Danish National Registry of Patients was used to estimate national incidence rates, and information on the date of death was obtained through linkage to the Civil Registration System. The study included 3351 patients with infective endocarditis from 1980 to 1997. The incidence rate for men increased from 4 to 6 per 100,000 person-years, and for women from 3 to 4 per 100,000 person-years. The mean incidence rate varied from 1.5 per 100,000 person-years in women younger than 50 y to 15.5 in men older than 70 y. The incidence increased in most age groups but was most pronounced in younger men. The overall 30-d case fatality rate of 23% increased with age and decreased with calendar time. The mean mortality rate was 0.94 per 100,000 y and decreased in the study period in most age groups. There was an increasing incidence of infective endocarditis and an improvement in the prognosis.     

The burden of large and small duct primary sclerosing cholangitis in adults and children: a population-based analysis

OBJECTIVES: The epidemiology of primary sclerosing cholangitis (PSC) has been incompletely assessed by population-based studies. We therefore conducted a population-based study to determine: (a) incidence rates of large and small duct PSC in adults and children, (b) the risk of inflammatory bowel disease on developing PSC, and (c) patterns of clinical presentation with the advent of magnetic resonance cholangiopancreatography (MRCP). METHODS: All residents of the Calgary Health Region diagnosed with PSC between 2000 and 2005 were identified by medical records, endoscopic, diagnostic imaging, and pathology databases. Demographic and clinical information were obtained. Incidence rates were determined and risks associated with PSC were reported as rate ratios (RR) with 95% confidence intervals (CI). RESULTS: Forty-nine PSC patients were identified for an age- and gender-adjusted annual incidence rate of 0.92 cases per 100,000 person-years. The incidence of small duct PSC was 0.15/100,000. In children the incidence rate was 0.23/100,000 compared with 1.11/100,000 in adults. PSC risk was similar in Crohn's disease (CD; RR 220.0, 95% CI 132.4-343.7) and ulcerative colitis (UC; RR 212.4, 95% CI 116.1-356.5). Autoimmune hepatitis overlap was noted in 10% of cases. MRCP diagnosed large duct PSC in one-third of cases. Delay in diagnosis was common (median 8.4 months). A minority had complications at diagnosis: cholangitis (6.1%), pancreatitis (4.1%), and cirrhosis (4.1%). CONCLUSIONS: Pediatric cases and small duct PSC are less common than adult large duct PSC. Surprisingly, the risk of developing PSC in UC and CD was similar. Autoimmune hepatitis overlap was noted in a significant minority of cases.