Primary biliary cholangiti

Xanthelasmas can be found at the corners of the eye or periorbitally, above all in cases of biliary cirrhosis due to primary biliary cholangitis (CDNC). (1) They display... 

Fig. 4.14 Xanthelasmas in biliary cirrhosis as a result of primary biliary cholangitis...

Elevation of serum copper is found in cholestasis, obstructive jaundice, primary biliary cholangitis, malignant tumours, kwashiorkor, exocrine pancreatic insufficiency, during the last trimenon of pregnancy and after administration of oestrogens. A decrease in serum copper is typical of Wilson s disease. In some rare cases, it is caused by familial benign hypocupraemia and nutritional deficiency in neonates. 

Primary biliary cholangitis or primary biliary cirrhosis are both characterized hy strong elevations of IgM. Only occasionally (in 20-30% of cases) is there also an elevation of IgG in immuno-logically and clinically more active forms. IgA values only show a slight increase during the late course. 

In primary biliary cholangitis, AMA were found in 95-100% of cases at high titres (>1 100). They usually... 

Paraneoplastic syndrome Parasitic infections Parenteral nutrition Postoperative cholestasis Primary biliary cholangitis Primary sclerosing cholangitis Protoporphyria Right ventricular failure Sepsis... 

Pruritus is accompanied by scratch marks (s. pp 85, 232), which the tormented patient inflicts, often with great intensity, on all accessible parts of the body (f as long as it bleeds, it doesn t itch any more ). Fresh scratch marks on the skin alternating with older, blood-encrusted sites are characteristic of the body surface of a patient suffering from chronic cholestasis, such as primary biliary cholangitis, (s. fig. 13.3)... 

Primary biliary cholangitis (78), primary sclerosing cholangitis... 

Even in the early stage of primary biliary cholangitis, immunological or scar-related atrophy of the small portal veins is evident. (78) In primary sclerosing cholangitis, these changes are only manifested once cirrhotic transformation has set in. (s. tab. 14.3)... 

Basedow hyperthyroidism BCG vaccine Erythematodes visceralis Granulomatosis infantisepticum Hypogammaglobulinaemia Polymyalgia rheumatica Primary biliary cholangitis Sarcoidosis Vasculitis... 

Fig. 32.8 Primary biliary cholangitis Map-like marking of the liver surface (stage I) still largely smooth surface (see light reflex), dark red/livid blue colouring tiger skin-like pattern in the irregular reddish areas...

Fig. 32.9 Primary biliary cholangitis with cirrhotic transformation. Well-rounded parenchymal residues surrounded by collagenous fibre tissue hardly recognizable bile ducts in the residual round cell-infiltrated portal fields (stages III—IV) (HE)...

The distinction between primary biliary cholangitis and primary or secondary sclerosing cholangitis by differential diagnosis may be made substantially more difficult at times owing to special courses of disease, autoimmune hepatitis or overlap syndromes. 

Immunologic reactions Primary biliary cholangitis, primary sclerosing cholangitis, overlap syndromes, allograft rejection, sarcoidosis, etc. are worth mentioning in this context. 

Kuntz, E. Mycotoxins — a primary immunological challenge involving molecular mimicry in primary biliary cholangitis (PBC) Hepatol. Rap. Lit. Rev. (Falk-Foundation) 1998 28 (3) IX-X... 

Primary biliary cholangitis The overlap of PBC with AIH reveals positivity of AMA, ANA and ASGPR as well as lower litres of LP and SMA. In approx. 20% of cases, AMA can be found in AIH. The frequency of this overlap syndrome is 5-10% (-15%). It was first detected in a child (12-year-old girl). (34) IgG and IgM fractions are simultaneously elevated. Histological findings can comprise the criteria of both PBC and CAH. Thus two different autoimmune diseases are present, whereby prognosis is determined by the degree of severity of AIH. This provides the indication for immunosuppressive therapy, (s. tab. 33.1) (s. p. 660)... 

Differential diagnosis The following conditions should be excluded (7.) alcohol-induced liver disease, (2.) primary biliary cholangitis (86), (3.) porphyria cutanea tarda (108), and (4.) autoimmune hepatitis (89) or autoimmune cholangitis (132) associated with anti-HCV. 

Autoimmune hepatitis (see chapter 33) Autoimmune cholangitis (see chapter 32) Primary biliary cholangitis (see chapter 32) Primary sclerosing cholangitis (see chapter 32) Wegener s granulomatosis... 

Rheumatic diseases can also cause direct or indirect liver damage. A common denominator is the frequent occurrence of HLA type B8. There is frequently a close association between rheumatic diseases or collagenoses and chronic viral hepatitis (B, C), autoimmune hepatitis, primary biliary cholangitis as well as cirrhosis. 

Reducing the cholesterol level and influencing lipid metabolic disturbances in primary biliary cholangitis (and other liver diseases accompanied by hyperlipidaemia)... 

The results obtained from the clinical use of colchicine are controversial. Good results in the prevention of cirrhosis in chronic hepatitis B and, when combined with UDCA, in primary biliary cholangitis are counterbalanced by unfavourable results in chronic hepatitis (283, 291), primary biliary cholangitis (281) and primary sclerosing cholangitis (285) as well as in alcoholic hepatitis. A beneficial effect of colchicine on hepatic fibrogenesis should be the subject of further debate, (s. pp 650, 658, 743)... 

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