A Case of Primary Biliary Cirrhosis which Developed Eight Years after Diagnosis of Systemic Lupus Erythematosus

Abstract

A 29-year-old Japanese female was diagnosed with systemic lupus erythematosus (SLE) at the age of 21 and went into remission after administration of prednisolone. Although no liver dysfunction had been observed upon diagnosis of SLE or during follow-up, an increase of hepatobiliary enzyme levels was seen eight years after the diagnosis of SLE. Antimitochondrial antibodies were positive. Cell infiltration around intrahepatic bile ducts and granuloma formation were observed in the liver. Therefore, she was diagnosed with primary biliary cirrhosis (PBC). Administration of ursodeoxycholic acid resulted in normalization of hepatobiliary enzyme levels. Development of PBC after SLE is extremely rare.

Concomitant Case of Primary Biliary Cirrhosis and Autoimmune Hemolityc Anemia Responding to Corticosteroid and Ursodeoxycholic Acid in Young Woman

“…Serum bilirubin levels have long been known to be important in assessing the prognosis of PBC, but confounding hemolysis may lead to erroneous conclusions about the severity of PBC. 2 At the same time, compared with primary AIHA, this PBC-related AIHA is lifethreatening but can be controlled. If no attention is paid to this condition, primary PBC will be ignored, as 50-60% of PBC patients are asymptomatic at diagnosis.…”

“…1,2,3 The disease is uncommon, with a reported prevalence between 19 and 402 cases per million. The vast majority (90%) of patients affected are women, with the usual time of diagnosis between 30 and 60 years.…”

A Literature Review of Concomitant Primary Biliary Cirrhosis and GravesÂ’ Disease

“…PBC is considered an autoimmune disease characterized by chronic progressive cholestasis with destruction of small intrahepatic bile ducts, particularly interlobular bile ducts [3][4][5][6]. PBC affects middleaged women (women in their fifth or sixth decade) more often than men (ratio of 9-10:1) [15,16].…”

“…The diagnosis of PBC is established when 2 of the following 3 objective criteria are present [14]: (1) elevated serum alkaline phosphatase (ALP) levels, (2) presence of anti-mitochondrial antibodies (AMAs), which are useful for the serological diagnosis of PBC (90%-95% of patients with PBC are AMA-positive) [6], and (3) liver histology findings.…”

“…PBC is characterized by chronic progressive cholestasis with destruction of small intrahepatic bile ducts, particularly interlobular bile ducts [3][4][5][6].…”