The Natural History and Prognosis of Primary Biliary Cirrhosis with Clinical Features of Autoimmune Hepatitis

Abstract

Although a variant of primary biliary cirrhosis (PBC) characterized by features of autoimmune hepatitis (AIH) has been recognized for many years, few studies with ample numbers of patients have focused on its natural history. This study aimed to clarify the natural history, prognosis, and response to therapy in a cohort of patients with PBC with AIH features. We retrospectively analyzed 277 PBC patients without AIH features and 46 PBC patients with AIH features seen between September 2004 and April 2014. The 5-year adverse outcome-free survival of PBC patients with AIH features was 58% compared to 81% in PBC patients without AIH features. Multivariate analysis in the patients with AIH features indicated that total bilirubin ≥ 2.70× the upper limit of normal predicted a poor prognosis (p = 0.008, relative risk 8.39, 95% confidence interval (CI) 1.73, 40.73). Combination therapy with ursodeoxycholic acid (UDCA) and immunosuppression provided better short-term responses in PBC patients with AIH features, defined by multiple criteria. Higher aspartate aminotransferase (AST) level at accession suggested better prognosis for PBC patients with AIH features while worse prognosis for PBC patients without AIH features. PBC patients with AIH features differ from those without AIH features in terms of natural history, prognostic indicators, and response to therapy.

Underestimated Male Prevalence of Primary Biliary Cholangitis in China: Results of a 16-yr cohort study involving 769 patients

“…for the first time proposed the underestimated male prevalence of PBC 15 , followed by 2 geoepidemiology researches in the West, where the female: male ratio could decrease to 1.6:1 from administrative databases in Sweden 5, 6 . The gender ratio in our study also corroborated the results of recent studies from South Korea and Japan (6.2:1 and 7.0:1, respectively) 16, 17 and is much lower than those in previous reports from China 9, 10 . This study is the largest of Chinese PBC patients and implies that the evolving trend of female to male incidence in China is similar to that observed in Western populations 5, 6 .…”

“…The disease spectrum often differs between the East and West (e.g., with regard to hepatitis B, hepatitis C, and autoimmune hepatitis; AIH). Recently, several studies have explored the clinicopathological characteristics of patients with PBC in China, and two reports found that the female:male ratio was 11.6:1 and 10.9:1, respectively 9, 10 . However, these studies in China have been performed in developed areas, such as Shanghai and Beijing, rather than developing areas.…”

Chinese consensus on the diagnosis and management of autoimmune hepatitis (2015)

“…Second‐line immunosuppressants (cyclosporine A, tacrolimus and MMF) induced biochemical remission in 54% of patients who did not respond to initial immunosuppressive agents. A recent study in China showed that a combination therapy of glucocorticoid and UDCA could significantly improve the short‐term prognosis of patients with overlap syndrome …”

Recent advances in the diagnosis and treatment of primary biliary cholangitis

“…Combination treatment with budesonide and UDCA was more efficacious than UDCA monotherapy for PBC/AIH overlap syndrome[159]. Furthermore, combination treatment with immunosuppression and UDCA offered better short-term responses in PBC/AIH overlap syndromes[160]. …”

“…Furthermore, the pooled 2 year rates of death, transplant, and progression of varicosities were 11.4%, 8.7% and 10.6%, respectively, in patients treated with placebo[165]. The natural history of PBC patients with AIH characteristics significantly differs from those without AIH characteristics[160]. In addition, although considered to possess a higher prevalence rate of AMAs, first-degree relatives of PBC patients have a lower risk of developing PBC over time, especially in those without baseline biochemical test evidence of intrahepatic cholestasis[166].…”