Gender as a negative prognostic factor in Primary biliary cholangitis

Introduction:

Primary Biliary Cholangitis (PBC) is a cholestatic liver disease of autoimmune origin disproportionally often affecting women. Here, we sought to evaluate how the clinical presentation and course of disease differs between male and female patients affected by PBC.

Patients and Methods:

We analyzed a cohort of 766 patients with a diagnosis of PBC and identified 49 male patients (6.4%) treated at our center between 2006 and 2017. We identified 2 age-matched female controls for each male patient. 49 male patients and 98 female patients were included in the further analysis. All patients were treated with UDCA. Patient charts were reviewed retrospectively.

Results:

Median age at diagnosis was 59 years, time between onset of symptoms and diagnosis was delayed in male patients (69 months compared to 32 months), p = 0.02. 17 male patients reported PBC-typical complaints compared to 70 female patients (p < 0.0001).

Whereas liver enzymes did not differ significantly prior to treatment, male patients showed a poorer treatment response to UDCA after one year (p = 0.03). Interestingly, IgM was signifcantly elevated in male patients (p = 0.01). Moreover, male patients scored significantly worse in the UK-PBC Score (p = 0.0002), and also in the GLOBE-Score (p < 0.0001).

Significantly more male patients received a liver biopsy (83.6% compared to 58%, p = 0.002): Male patients tended to have less hepatitis activity compared to female patients but showed a significantly higher grade of fibrosis. Three male patients with liver cirrhosis developed hepatocellular carcinoma within the study period compared to one female patient in the whole cohort of more than 750 female patients.

Discussion:

Male PBC patients report fewer symptoms than female patients but tend to develop complications from PBC more often. The higher extent of fibrosis is most likely due to the delayed diagnosis. As PBC runs a more complicated course in male patients compared to female patients, more intensive follow-up and especially regular ultrasound examinations are warranted in male patients.