Liver transplantation (LT) is an established therapy for patients with end-stage primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). In this report, we describe the health status and quality of life (QOL) in patients with these cholestatic liver diseases before and after LT. A QOL questionnaire was completed by 157 adult patients with PBC or PSC before and 1 year after liver transplantation at the Mayo Clinic or Baylor University Medical Center. This questionnaire measured four aspects of QOL, including symptoms; physical, social, and emotional functioning; health perceptions; and overall QOL. Changes in these QOL parameters before and after LT were described, and regression analysis was used to assess the relationships between clinical and QOL factors. There were no differences in QOL parameters between patients with PBC and PSC. QOL following transplantation was substantially better than before transplantation. This was observed in all four aspects of QOL. The degree of improvement as measured by effect size (difference in mean scores divided by the pretransplantation standard deviation) was 0.53 for symptoms (P F .01), 1.16 for function (P F .01), 2.37 for health satisfaction (P F .01), and 1.16 for overall QOL (P F Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are cholestatic liver diseases of unknown etiology with distinctive clinical and epidemiological features. These diseases are characterized by a slow and usually progressive course that, over time, leads to cirrhosis, portal hypertension, and, eventually, liver failure. Patients with liver failure from end-stage PBC or PSC are potential candidates for orthotopic liver transplantation (LT). Between 1988 and 1994, these patients accounted for approximately 17% of LT recipients. 1 Following LT, these patients have an improved prognosis, with 2-year survival rates reported to be over 90%. 2 It is not known how rapidly or completely LT reverses the devastating symptoms of severe PBC or PSC, or how well these patients adapt to the drug regimens necessary to maintain their grafts. Information about these benefits and consequences of LT taken from the patient' s perspective can improve clinicians' understanding of the recovery process and enable clinicians to better prepare these patients and their families for LT. Furthermore, this information may identify important issues for long-term survivors and contribute to the evaluation of new therapies to enhance LT outcomes. Information of this type, about the effects of an illness and its consequent therapy on a patient' s health and well-being as perceived by the patient, is termed quality of life (QOL), or more specifically, health-related QOL. 3 While a number of studies have described the QOL outcomes of heterogeneous series of LT recipients, 4-8 QOL findings specific to patients with cholestatic liver disorders are very limited. 9 Patients with PBC or PSC form a distinct subgroup of LT patients in terms of their demographics, symptomatology, and complications of...