Concurrent Occurrence of Primary Biliary Cirrhosis and Rheumatoid Arthritis

Abstract

Primary biliary cirrhosis (PBC) is an autoimmune cholestatic disorder of the liver. A diagnostic serum marker for PBC is an anti-mitochondrial antibody. Most prominent histologic findings of PBC are portal inflammation and destruction of interlobular bile ducts. The PBC occurs only in 40 to 400 individuals per million in the general population. About 1.8 - 5.6% of individuals with this rare disorder have rheumatoid arthritis (RA). This case report describes a 56-year-old female with concurrent rheumatoid arthritis and primary biliary cirrhosis. The patients with RA are at higher risk of developing PBC compared to the general population. Thus, abnormal liver function test in the patients with RA, especially in the absence of alternative cause, warrants thorough investigation for PBC. Early diagnosis and treatment will improve the outcome of patients who develop PBC.

Autoimmune Liver Diseases and Rheumatoid Arthritis—Is There an Etiopathogenic Link?

“…Genetic investigations were carried out in an attempt to discover a connection between RA and PBC, revealing some common genes, such as histocompatibility complex, class II, DQ beta 1 (HLA-DQB1), membrane metallo-endopeptidase-like 1 (MMEL1), interferon regulatory factor 5 (IRF5), cytotoxic Tlymphocyte-associated protein 4 (CTLA4), signal transducer and activator of transcription 4 (STAT4), and CXC chemokine receptor 5 (CXCR5). This shared genetic trait predisposes individuals with RA to the development of PBC, indicating a genetic link between the two conditions [11,36,37].…”

“…PBC is a liver condition that is histologically identified by chronic non-suppurative damage and gradual loss of intrahepatic small bile ducts, leading to fibrosis and, in the later stages, eventual cirrhosis [34][35][36]. The condition primarily affects women in their middle age [37].…”

“…While PBC can independently result in articular manifestations, primarily arthralgia, it is not linked to synovitis or X-ray modifications [35]. Antimitochondrial antibodies (AMAs) can be identified in the serum, typically detected at titers of 1/40 or higher, and are considered the characteristic feature of the disease [34][35][36]. These antibodies demonstrate a specificity exceeding 99% and a sensitivity of approximately 92% [39,40].…”

“…PBC is the most common coexisting AILD in RA, with a prevalence of from 3.8% to 6.3% [6,15,30,47,48], while the occurrence of RA in PBC has been reported to be from 1.8% to 13% [49][50][51]. Pak suggested that patients diagnosed with RA face an elevated risk of developing PBC compared to the population at large and that, consequently, when patients with RA exhibit irregularities in liver function tests, particularly in the absence of alternative causative factors, a comprehensive investigation for PBC is warranted [36]. Reports indicate that the majority of individuals with both RA and PBC typically experience the onset of RA several years before the development of PBC [36].…”

“…Pak suggested that patients diagnosed with RA face an elevated risk of developing PBC compared to the population at large and that, consequently, when patients with RA exhibit irregularities in liver function tests, particularly in the absence of alternative causative factors, a comprehensive investigation for PBC is warranted [36]. Reports indicate that the majority of individuals with both RA and PBC typically experience the onset of RA several years before the development of PBC [36]. In Siegel et al's study, the majority of the patients (n = 17) received the diagnosis of RA first, with an average lead time of 11.8 years before the diagnosis of PBC (range 2-27).…”