2793 Primary Biliary Cholangitis (PBC): Any Differences Between Males and Females?

Abstract

INTRODUCTION: PBC (primary biliary cholangitis) is a cholestatic autoimmune liver disease characterized by destruction of small intrahepatic bile ducts. PBC predominantly affects females but has also been reported in males. Characteristics of PBC in males have not been well described. We aimed to determine the incidence, characteristics, and prognosis of male patients with PBC as compared to females. METHODS: We performed a retrospective chart review of all patients with biopsy proven PBC seen at our large tertiary care hospital. We collected data on demographics, clinical and laboratory variables, mortality, and liver transplantation if any. Kaplan-Meier analysis was used to determine time to development of hepatic decompensation in patients with PBC, stratified by gender. RESULTS: We included 290 patients with biopsy-proven PBC, 254 (88%) were females and 36 (12%) were males. Mean age at diagnosis of PBC in males versus females was 50 ± 17 vs 54 ± 12 years, respectively (P = 0.09). Body mass index (P = 0.3) and presence of Diabetes (P = 0.06) were comparable between males and females. Mean alkaline phosphatase was similar between both groups at diagnosis, 334 U/L vs 320 U/L (P = 0.61). At the time of diagnosis, 31% of Males had advanced hepatic fibrosis (F3-F4) as compared to 16% of females (P = 0.09) (Table). AMA negative PBC was noted in 16% of females versus 0% of males. Disease progression was higher in males; portal hypertension developed in 42% of males vs 16% in females (P < 0.001), and decompensated cirrhosis was noted in 33% of males versus 10% of females (P < 0.001). Kaplan Meier time to hepatic decompensation showed increased risk of development of hepatic decompensation in males as compared to females (P < 0.001) (see figure). Bone disease was higher in females (osteopenia 22.5% and 6% osteoporosis) than males (12.5% and 3%, respectively). CONCLUSION: Male patients with PBC may have advanced fibrosis at diagnosis as compared to females. Additionally, male PBC patients seem to have a more aggressive course as they are more likely to develop portal hypertension and hepatic decompensation as compared to female patients with PBC, despite comparable age, and alkaline phosphatase level at the time of diagnosis. Bone disease was more common in the females as compared to males.

Reasons why women are more likely to develop primary biliary cholangitis

“…Women are undoubtedly more susceptible to autoimmunity than men and are more likely to suffer from symptoms of pruritus and skin pigmentation in PBC, with pruritus occurring more frequently while taking oral contraceptives and during.pregnancy [ 13 , 14 ]. Clinical studies have also found that PBC patients are more likely to undergo hysterectomy for menstrual irregularities and that PBC patients are more likely to receive hormone replacement therapy [ 15 , 16 ].…”

Sex differences in chronic liver disease and benign liver lesions

“…In terms of diagnostics, anti-mitochondrial antibody is absent in 15% of women with PBC while anti-mitochondrial antibody negativity typically excludes a diagnosis of PBC in men. 88 Women are typically younger and more symptomatic than men at diagnosis. Women report more fatigue and pruritis, which may relate to hormonal differences.…”