Patients with asymptomatic primary sclerosing cholangitis frequently have progressive disease

Michael K. Porayko, Russell H. Wiesner, Nicholas F La Russo, Jurgen Ludwig, Robert L. MacCarty, Betty L. Steiner, Colleen K. Twomey, Alan R. Zinsmeister

Research output: Contribution to journalArticle

114 Citations (Scopus)

Abstract

We identified and analyzed 45 patients with asymptomatic primary sclerosing cholangitis to better understand the natural history of this disease. Disease progression was monitored at regular intervals for the development of symptoms and physical signs as well as changes in liver biochemistry, cholangiography, and liver histology. During a median follow-up of 75.2 mo, 34 patients (76%) had evidence of disease progression. Fourteen patients (31%) developed liver failure which resulted in death or referral for liver transplantation. For patients with primary sclerosing cholangitis, survival curves computed using the Kaplan-Meier method were significantly worse than expected when compared to age-, sex-, and race-specific survival rates for the United States north central population (p < 0.001). These findings indicate that primary sclerosing cholangitis is generally a progressive disease with considerable morbidity and mortality even when detected before the onset of symptoms.

Original languageEnglish (US)
Pages (from-to)1594-1602
Number of pages9
JournalGastroenterology
Volume98
Issue number6
StatePublished - 1990

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Sclerosing Cholangitis
Disease Progression
Cholangiography
Liver
Liver Failure
Biochemistry
Liver Transplantation
Signs and Symptoms
Histology
Referral and Consultation
Survival Rate
Morbidity
Survival
Mortality
Population

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Porayko, M. K., Wiesner, R. H., La Russo, N. F., Ludwig, J., MacCarty, R. L., Steiner, B. L., ... Zinsmeister, A. R. (1990). Patients with asymptomatic primary sclerosing cholangitis frequently have progressive disease. Gastroenterology, 98(6), 1594-1602.

Patients with asymptomatic primary sclerosing cholangitis frequently have progressive disease. / Porayko, Michael K.; Wiesner, Russell H.; La Russo, Nicholas F; Ludwig, Jurgen; MacCarty, Robert L.; Steiner, Betty L.; Twomey, Colleen K.; Zinsmeister, Alan R.

In: Gastroenterology, Vol. 98, No. 6, 1990, p. 1594-1602.

Research output: Contribution to journalArticle

Porayko, MK, Wiesner, RH, La Russo, NF, Ludwig, J, MacCarty, RL, Steiner, BL, Twomey, CK & Zinsmeister, AR 1990, 'Patients with asymptomatic primary sclerosing cholangitis frequently have progressive disease', Gastroenterology, vol. 98, no. 6, pp. 1594-1602.
Porayko MK, Wiesner RH, La Russo NF, Ludwig J, MacCarty RL, Steiner BL et al. Patients with asymptomatic primary sclerosing cholangitis frequently have progressive disease. Gastroenterology. 1990;98(6):1594-1602.
Porayko, Michael K. ; Wiesner, Russell H. ; La Russo, Nicholas F ; Ludwig, Jurgen ; MacCarty, Robert L. ; Steiner, Betty L. ; Twomey, Colleen K. ; Zinsmeister, Alan R. / Patients with asymptomatic primary sclerosing cholangitis frequently have progressive disease. In: Gastroenterology. 1990 ; Vol. 98, No. 6. pp. 1594-1602.
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