Autoantibodies in primary sclerosing cholangitis

Abstract

The aetiology of primary sclerosing cholangitis (PSC) is not known and controversy exists as to whether PSC should be denominated an autoimmune disease. A large number of autoantibodies have been detected in PSC patients, but the specificity of these antibodies is generally low, and the frequencies vary largely between different studies. The presence of autoantibodies in PSC may be the result of a nonspecific dysregulation of the immune system, but the literature in PSC points to the possible presence of specific antibody targets in the biliary epithelium and in neutrophil granulocytes. The present review aims to give an overview of the studies of autoantibodies in PSC, with a particular emphasis on the prevalence, clinical relevance and possible pathogenetic importance of each individual marker.

Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

“…A key role of genetic predisposition is supported by studies that have documented familial occurrence [30] and association of the disease with specific haplotypes [31]. In addition, the existence of a significant immunologic component is supported by the high prevalence of a variety of autoantibodies in PSC [32] and by its strong association with IBD and other autoimmune disorders. A potential role of colonic inflammation and bacteria or bacterial toxin in the pathogenesis of PSC has also been proposed; however, no evidence has clearly confirmed this hypothesis.…”

“…Laboratory evaluation typically demonstrates a cholestatic biochemical picture. Several autoantibodies may be present in patients with PSC [32], but they lack specificity and are of limited clinical applicability.…”

Genetic Risk and the Development of Autoimmune Liver Disease

“…no single specificity occurring, rather profiles). Multiple autoantibodies are already known in autoimmune liver diseases [66][67][68] ; however, apart from a general association between the ancestral haplotype 8.1 and autoantibody generation that applies to any disease [69,70] , the relationship between antibody specificities and HLA associations remains obscure. Although speculative, an intriguing parallel may again be drawn to celiac disease, where autoantibodies against tissue transglutaminase directly link to the gluten reactivity, which again links to the HLA specificity profile of celiac disease patients [61,71] .…”

Akutes Nierenversagen bei einem lebertransplantierten Patienten

“…Der derzeitigen Auffassung nach spielen atypische ANCA keine pathogene Rolle in der Krankheitsentstehung systemischer Vaskulitiden. Im Gegensatz dazu ist das Auftreten von typischen MPO-(0-33%) oder PR3-AN-CA (0-44%) deutlich seltener [3,4]. Diese statistischen Wahrscheinlichkeiten sind im Einzelfall wenig hilfreich.…”