Abstract
Objectives Diagnosis and treatment of primary biliary cholangitis (PBC) are often complicated by hepatic and/or extrahepatic manifestations, which in turn affect the natural course and prognosis of PBC. This study evaluated the clinical characteristics and prognosis of PBC co-occurring with intrahepatic and extrahepatic autoimmune disease (AID). Methods Clinical data of patients with PBC who were admitted to the Beijing Ditan Hospital from September 2008 to December 2014 were retrospectively reviewed, assessed for other autoimmune diseases, and analyzed statistically. All patients received ursodeoxycholic acid (UDCA) treatment. Results Data from 505 patients were evaluated. Approximately 35.0% of patients had at least one additional AID. AIDs included Sjögren's syndrome (SS; 26.3%), autoimmune hepatitis (AIH; 7.1%), rheumatoid arthritis (RA; 1.4%), hypothyroidism (0.8%), Graves's thyroiditis (0.6%), systemic lupus erythematosus (SLE; 0.4%), and Hashimoto's thyroiditis (0.2%). No differences in response rates of UDCA were found between the PBC group and the PBC-SS group or PBC complicated with AID group (both P > 0.05). White blood cell (WBC, RR = 1.072, 95% CI: 1.016–1.130, P=0.011), platelet counts (PLT, RR = 0.995, 95% CI: 0.992–0.998, P=0.003), and prothrombin time and international normalized ratio (PT/INR, RR = 1.799, 95% CI: 1.010–3.206, P=0.046) were independent prognostic factors in patients with PBC. The overall survival time of patients in PBC-AIH and PBC-SS groups was shorter than that of those with PBC (P < 0.001). Conclusions AIH was the most common in hepatic comorbidity. SS was the most frequent extrahepatic comorbidity. WBC, PLT, and PT/INR were independent prognostic factors in patients with PBC. AID coexisted with PBC impaired patients' survival.
Highlights
Primary biliary cholangitis (PBC) is a granulomatous autoimmune disease (AID) characterized by portal tracts inflammation due to lymphocyte infiltration, progressive loss of bile ducts resulting in chronic cholestasis and subsequent fibrosis, cirrhosis [1], and eventually liver failure [2]. e most common manifestations of PBC are fatigue and pruritus. e prevalence of PBC has been shown to vary globally and regionally, with prevalence rates ranging from 19 to 402 cases per million individuals [3]
PBC is an immune-mediated epithelitis with complex pathogenetic mechanisms and often concomitantly occurs with autoimmune hepatitis (AIH), systemic lupus erythematosus (SLE), undifferentiated and mixed connective tissue diseases, chronic thyroiditis, rheumatoid arthritis (RA), systemic sclerosis (SSc), and other extrahepatic autoimmune (EHA) conditions such as Sjogren’s syndrome (SS) [4]. ese coexisting conditions frequently increase the difficulty in making a diagnosis and treating the disease
PBC was diagnosed based on the American Association for the Study of Liver Diseases (AASLD) 2009 Practice Guidelines [11]. e diagnosis of PBC was established if patients showed at least two of the following criteria: (1) presence of serum antimitochondrial antibody (AMA) titer greater than 1 : 40 (“AMA-positive”), (2) presence of abnormal alkaline phosphatase (ALP) levels, and (3) presence of PBC-compatible histopathology. e AMA-negative variant was diagnosed in cases with abnormal alkaline phosphatase levels, an antinuclear antibody (ANA) positivity of at least 1 : 40, and a liver histology compatible with PBC
Summary
Primary biliary cholangitis (PBC) is a granulomatous autoimmune disease (AID) characterized by portal tracts inflammation due to lymphocyte infiltration, progressive loss of bile ducts resulting in chronic cholestasis and subsequent fibrosis, cirrhosis [1], and eventually liver failure [2]. e most common manifestations of PBC are fatigue and pruritus. e prevalence of PBC has been shown to vary globally and regionally, with prevalence rates ranging from 19 to 402 cases per million individuals [3]. E prevalence of PBC has been shown to vary globally and regionally, with prevalence rates ranging from 19 to 402 cases per million individuals [3]. PBC is an immune-mediated epithelitis with complex pathogenetic mechanisms and often concomitantly occurs with autoimmune hepatitis (AIH), systemic lupus erythematosus (SLE), undifferentiated and mixed connective tissue diseases, chronic thyroiditis, rheumatoid arthritis (RA), systemic sclerosis (SSc), and other extrahepatic autoimmune (EHA) conditions such as Sjogren’s syndrome (SS) [4]. Canadian Journal of Gastroenterology and Hepatology ere is a significant variability in the reported incidence rates and types of PBC co-occurring with other autoimmune diseases across different countries and regions. While in China Wang et al showed that PBC overlapped with connective tissue diseases in about 46.6% of patients [7], other studies have shown that these probabilities may be underestimated [8]
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