Abstract
The etiology of the autoimmune liver disease primary biliary cirrhosis (PBC) remains
largely unresolved, owing in large part to the complexity of interaction between environmental
and genetic contributors underlying disease development. Observations of disease clustering,
differences in geographical prevalence, and seasonality of diagnosis rates suggest
the environmental component to PBC is strong, and epidemiological studies have consistently
found cigarette smoking and history of urinary tract infection to be associated with
PBC. Current evidence implicates molecular mimicry as a primary mechanism driving
loss of tolerance and subsequent autoimmunity in PBC, yet other environmentally influenced
disease processes are likely to be involved in pathogenesis. In this review, the authors
provide an overview of current findings and touch on potential mechanisms behind the
environmental component of PBC.
Keywords
autoimmunity - environment - primary biliary cirrhosis
