Primary sclerosing cholangitis epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dima Nimri, M.D. [2]

Overview

Primary sclerosing cholangitis is a relatively rare disease, with the incidence ranging from 0 to 1.3 per 100,000 cases and prevalence of 0 to 16.2 per 100,000 cases. The disease primarily affects young adult males and the mean age at diagnosis is 41 years.

Epidemiology

The majority of data on the incidence and prevalence of primary sclerosing cholangitis are from North America and Europe. The global incidence of the disease is hard to estimate due to the paucity of studies done in developing countries. Because inflammatory bowel disease is much more prevalent in North America and Europe, and it is strongly associated with primary sclerosing cholangitis, it is thought that data from these regions overestimate the global incidence and prevalence of the disease.[1][2][3][4][5]

Incidence

The incidence of primary sclerosing cholangitis ranges from 0 to 1.3 per 100,000 cases.[1][2]

Prevalence

The prevalence of primary sclerosing cholangitis ranges from 0 to 16.2 per 100,000 cases.[1][2]

Demographics

Age

Primary sclerosing cholangitis is most prevalent between the ages of 35-47 years, with the mean age at diagnosis being 41.[1][2]

Gender

Primary sclerosing cholangitis is more prevalent in males than females, with a male-to-female ratio of 1.70.[1]

References

  1. 1.0 1.1 1.2 1.3 1.4 Molodecky NA, Kareemi H, Parab R, Barkema HW, Quan H, Myers RP, Kaplan GG (2011). "Incidence of primary sclerosing cholangitis: a systematic review and meta-analysis". Hepatology. 53 (5): 1590–9. doi:10.1002/hep.24247. PMID 21351115.
  2. 2.0 2.1 2.2 2.3 Lazaridis KN, LaRusso NF (2016). "Primary Sclerosing Cholangitis". N. Engl. J. Med. 375 (12): 1161–70. doi:10.1056/NEJMra1506330. PMID 27653566.
  3. Bambha K, Kim WR, Talwalkar J, Torgerson H, Benson JT, Therneau TM, Loftus EV, Yawn BP, Dickson ER, Melton LJ (2003). "Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a United States community". Gastroenterology. 125 (5): 1364–9. PMID 14598252.
  4. Boonstra K, Beuers U, Ponsioen CY (2012). "Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review". J. Hepatol. 56 (5): 1181–8. doi:10.1016/j.jhep.2011.10.025. PMID 22245904.
  5. Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H (1998). "Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population". Scand. J. Gastroenterol. 33 (1): 99–103. PMID 9489916.


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