Rapid-onset Primary Biliary Cirrhosis Resembling Drug-induced Liver Injury

Sohda, Tetsuro; Shiga, Hiroshi; Nakane, Hidetoshi; Nishizawa, Shinya; Yoshikane, Makoto; Anan, Akira; Suzuki, Norihisa; Irie, Makoto; Iwata, Kazuo; Watanabe, Hiroshi; Sakisaka, Shotaro

doi:10.2169/internalmedicine.44.1051

Cited by 6 publications

(5 citation statements)

References 24 publications

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“…This study found that mixed type DILI and PBC had significant differences in elevated transaminases and bilirubin, while cholestatic type DILI and PBC had no significant differences in all biochemical indexes except total protein. Given that DILI patients may also test positive for autoantibodies and there have been reports that DILI can progress into PBC and AIH (autoimmune hepatitis)-PBC overlap syndrome[14] or PBC was misdiagnosed as DILI[15], clinicians should pay more attention to the differential diagnosis of DILI from PBC in clinical practice.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of drug-induced liver injury and primary biliary cirrhosis

Yang¹,

Yu²,

Jin³

et al. 2016

WJG

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AIMTo summarize and compare the clinical characteristics of drug-induced liver injury (DILI) and primary biliary cirrhosis (PBC).METHODSA total of 124 patients with DILI and 116 patients with PBC treated at Shengjing Hospital Affiliated to China Medical University from 2005 to 2013 were included. Demographic data (sex and age), biochemical indexes (total protein, albumin, alanine aminotransferase, aspartate aminotransferase, total bilirubin, direct bilirubin, indirect bilirubin, alkaline phosphatase, and gamma glutamyltransferase), immunological indexes [immunoglobulin (Ig) A, IgG, IgM, antinuclear antibody, anti-smooth muscle antibody, anti-mitochondrial antibody, and anti-mitochondrial antibodies] and pathological findings were compared in PBC patients, untyped DILI patients and patients with different types of DILI (hepatocellular type, cholestatic type and mixed type).RESULTSThere were significant differences in age and gender distribution between DILI patients and PBC patients. Biochemical indexes (except ALB), immunological indexes, positive rates of autoantibodies (except SMA), and number of cases of patients with different ANA titers (except the group at a titer of 1:10000) significantly differed between DILI patients and PBC patients. Biochemical indexes, immunological indexes, and positive rate of autoantibodies were not quite similar in different types of DILI. PBC was histologically characterized mainly by edematous degeneration of hepatocytes (n = 30), inflammatory cell infiltration around bile ducts (n = 29), and atypical hyperplasia of small bile ducts (n = 28). DILI manifested mainly as fatty degeneration of hepatocytes (n = 15) and spotty necrosis or loss of hepatocytes (n = 14).CONCLUSIONAlthough DILI and PBC share some similar laboratory tests (biochemical and immunological indexes) and pathological findings, they also show some distinct characteristics, which are helpful to the differential diagnosis of the two diseases.

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Section: Discussionmentioning

confidence: 99%

Clinical characteristics of drug-induced liver injury and primary biliary cirrhosis

Yang¹,

Yu²,

Jin³

et al. 2016

WJG

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“…In our case, the patients showed rapid elevation in ALT as well as ALP and γ GTP. Sohda et al previously reported a rare case of rapid‐onset PBC. In their case, drug‐induced liver injury (DILI) and overlap syndrome between PBC and auto immune hepatitis (AIH) were suspected at first.…”

Section: Discussionmentioning

confidence: 99%

A case of primary biliary cirrhosis in a patient with rheumatoid arthritis

Bekki

Bae

Yoshizawa

et al. 2015

Clinical Case Reports

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“…Interestingly, one patient (patient 3) presented with an acute hepatitis-like condition with markedly elevated serum aminotransferases. In PBC, acute presentations have very rarely been reported[ 11 ]. Because acute presentations of an overlapping syndrome, i.e ., autoimmune hepatitis, are common, it has previously been suggested that autoimmune hepatitis might exhibit overlap with the early stage of PBC in PBC patients with acute presentations[ 12 ].…”

Section: Discussionmentioning

confidence: 99%

Evidence from a familial case suggests maternal inheritance of primary biliary cholangitis

Shin

Moh

Woo

et al. 2017

WJG

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Primary biliary cholangitis (PBC) is an idiopathic autoimmune liver disease characterized by chronic cholestasis and destruction of the intrahepatic bile ducts. Similar to other autoimmune diseases, the pathogenesis of PBC is considered to be a complex etiologic phenomenon involving the interaction of genetic and environmental factors. Although a number of common variants associated with PBC have been reported from genome-wide association studies, a precise genetic mechanism underlying PBC has yet to be identified. Here, we describe a family with four sisters who were diagnosed with PBC. After the diagnosis of the index patient who was in an advanced stage of PBC, one sister presented with acute hepatitis, and two sisters were subsequently diagnosed with PBC. Notably, one half-sister with a different mother exhibited no evidence of PBC following clinical investigation. Our report suggests the possibility of a maternal inheritance of PBC susceptibility. Moreover, judging from the high-penetrance of the disease observed in this family, we inferred that a pathogenic genetic variant might be the cause of PBC development. We describe a family that exhibited diverse clinical presentations of PBC that included asymptomatic stages with mildly increased liver enzyme levels and symptomatic stages with acute hepatitis or advanced liver fibrosis. Additional studies are needed to investigate the role of genetic factors in the pathogenesis of this rare autoimmune disease.

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Rapid-onset Primary Biliary Cirrhosis Resembling Drug-induced Liver Injury

Cited by 6 publications

References 24 publications

Clinical characteristics of drug-induced liver injury and primary biliary cirrhosis

Clinical characteristics of drug-induced liver injury and primary biliary cirrhosis

A case of primary biliary cirrhosis in a patient with rheumatoid arthritis

Evidence from a familial case suggests maternal inheritance of primary biliary cholangitis

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