Systemic lupus erythematosus (SLE) and primary biliary cirrhosis (PBC)
are distinct clinical disorders which rarely occur in the same patien
t. We report on a 65-year-old woman with coexistence of both condition
s. Diagnosis of SLE was ascertained by the presence of seven ACR crite
ria (cutaneous lesions, photosensitivity, antinuclear and anti-double-
stranded-DNA antibodies, pancytopenia, arthritis, oral lesions). PBC w
as disclosed by clinical investigation, liver histology and highly pos
itive antimitochondrial M2 antibodies. The most important differential
diagnoses of lupus hepatitis are PBC and autoimmune hepatitis. Diagno
stic criteria for these conditions are discussed, and previous reports
on overlap between SLE and PBC are reviewed.