Primary biliary cirrhosis: Clinical and laboratory criteria for its diagnosis

Abstract

Primary biliary cirrhosis (PBC) is a chronic progressive cholestatic granulomatous, and destructive inflammatory lesion of small intralobular and septal bile ducts, which is likely to be caused by an autoimmune mechanism with a the presence of serum antimitochondrial antibodies and a potential tendency to progress to cirrhosis. Despite the fact that the etiology of this disease has been unknown so far, there has been a considerable body of scientific evidence that can reveal the clinical and laboratory signs of PBC and the individual components of its pathogenesis and elaborate diagnostic criteria for the disease and its symptomatic therapy. Deficiencies in autoimmune tolerance are critical factors for the initiation and perpetuation of the disease. The purpose of this review is to summarize the data available in the literature and the author's findings on clinical and laboratory criteria for the diagnosis of PBC. This review describes the major clinical manifestations of the disease and the mechanisms of its development. It presents the immunological, biochemical, and morphological signs of PBC and their significance for its diagnosis. A great deal of novel scientific evidence for the problem of PBC has been accumulated. However, the inadequate efficiency of therapy for the disease lends impetus to the quest for its etiological factors and to further investigations of its pathogenetic mechanisms and, on this basis, to searches for new methods for its early diagnosis.

Infliximab as a treatment option for patients with rheumatoid arthritis and primary biliary cirrhosis

“…These findings were consistent with PBC stage III according to the Ludwig's classification [13]. On the basis of the histological features, the presence of AMA and the cholestatic biochemical pattern, patient was diagnosed with PBC [1,14]. Treatment with ursodeoxycholic acid 13 mg/kg/day was started.…”

“…The typical serological feature of the disease is the presence of the antimitochondrial antibodies (AMA), detected at titers of 1/40 or greater [1,2]. Autoimmune diseases-predominantly systemic sclerosis-are associated with PBC; however, autoimmune liver abnormalities may occur-to a much lesser extend-in patients with rheumatoid arthritis (RA) [1][2][3][4][5][6][7][8]. Both PBC and RA are considered immunologically mediated diseases in which the proinflammatory cytokine tumor necrosis factor alpha (TNFa) has demonstrated to play a prominent pathogenetic role [2,9].…”

Reduction and stabilization of bilirubin with obeticholic acid treatment in patients with primary biliary cholangitis

“…In this post hoc analysis, patients in the POISE intent‐to‐treat population (N = 216) were pooled across treatment groups and equally divided into quartiles by baseline direct bilirubin levels (as this is the main elevated component in PBC) to investigate whether there was a relationship between baseline direct bilirubin level and laboratory measures throughout the course of the study 20,25 . Efficacy and safety analyses were evaluated by direct baseline bilirubin quartiles and treatment groups (Table 1).…”

“…These guidelines state that direct bilirubin elevations, and not indirect elevations, are often present in cholestatic disorders with impairment in bile flow 22 . In PBC, the levels of bilirubin are elevated mainly by its direct fraction 20,23. Taken together, these data and recommendations suggest that direct bilirubin may be a more sensitive marker for PBC severity than total bilirubin.…”

“…Most studies and prognostic scores report on only total bilirubin, although a key disease‐related component of total bilirubin is direct or conjugated bilirubin 6‐7,18‐20 . Tahtaci et al evaluated various haematological and biochemical parameters in patients with advanced or early‐stage PBC and found that, of total and direct bilirubin, only direct bilirubin level was significantly higher in patients with late‐stage disease 21 .…”