Primary biliary cirrhosis presented as peripheral eosinophilia in asymptomatic women with or without elevated alkaline phosphatase.

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by the destruction of biliary epithelial cells, presumably by autoimmune mechanism(s). Although lymphocytes play a pivotal role in the pathogenesis of PBC, the possible involvement of eosinophils has also been suggested. Recent studies have shown that eosinophilia possibly occurs in the peripheral blood of PBC patients. We present four cases of asymptomatic middle-aged women with moderate-to-high eosinophilia observed during random investigation. Alkaline phosphatase (ALP) was increased in two of them. As a result of clinical and laboratory evaluations the early stages of PBC were diagnosed in all the patients, as attested by the detection of high titres of anti-mitochondrial antibodies and the characteristic lesions on liver biopsies. Liver function tests and eosinophils returned within normal limits after 2 months of treatment with ursodeoxycholic acid, suggesting that its potential immunomodulatory actions may extend to eosinophils. Our report further supports the possibility that eosinophilia may occur in PBC, especially in its early stages. From the clinical point of view, we believe that PBC should be considered in the differential diagnosis of eosinophilia with an otherwise unknown origin. In particular, PBC should be suspected in a patient when other causes of eosinophilia have been excluded, irrespective of the presence or absence of symptoms, or the presence or absence of elevated ALP. In such cases further evaluation for anti-mitochondrial antibodies should be done. These observations might assist the development of future therapeutic concepts in the management of PBC, at least for patients in early stages of the disease.

Increased incidence of anti-LKM autoantibodies in a consecutive cohort of hepatitis C patients from central Greece.

OBJECTIVES: In Greece, there are insufficient data regarding the presence of non-organ and liver-related autoantibodies in hepatitis C patients. This study in a consecutive cohort of 39 such patients from central Greece investigates (1) the prevalence of non-organ and liver-related autoantibodies, and (2) the reactivity of anti-liver-kidney microsomal type 1 antibodies (in the case of positivity with at least one of the methods used) against their molecularly defined antigens. DESIGN: All serum samples were tested by standard and molecular assays for the presence of anti-nuclear antibodies, smooth muscle antibodies, anti-liver-kidney microsomal type 1 antibodies, antibodies against parietal cells, anti-CYP2A6, anti-CYP1A2 and anti-CYP2D6 autoantibodies. METHODS: Indirect immunofluorescence, competitive enzyme-linked immunosorbent assays, immunoblotting and novel radioligand assays based on immunoprecipitation of [35S]-methionine labelled recombinant CYP2A6, CYP1A2 and CYP2D6 His-taq fusion proteins produced by in vitro transcription/translation were used. RESULTS: Seven out of 39 patients (17.9%) tested positive for smooth muscle antibodies, 2/39 (5.1%) tested positive for anti-nuclear antibodies, 1/39 (2.5%) tested positive for parietal cell antibodies, and 4/39 (10.3%) were found to be anti-liver-kidney microsomal positive (with at least one of the methods used). All sera were negative for anti-CYP2A6 and anti-CYP1A2 autoantibodies. Three out of four anti-liver-kidney microsomal positive samples had the typical liver-kidney microsomal staining pattern shown by indirect immunofluorescence. However, none tested positive for anti-CYP2D6 autoantibodies using the competitive CYP2D6 enzyme-linked immunosorbent assay, the specific CYP2D6 radioligand assay, and western blot using either human microsomes or recombinant CYP2D6. The fourth patient tested negative for anti-liver-kidney autoantibodies by either indirect immunofluorescence or the competitive enzyme-linked immunosorbent assay, but was repeatedly positive for anti-CYP2D6 autoantibodies by the sensitive and specific radioligand assay. Western blot experiments using human microsomes in this patient serum revealed two bands of 50 kDa and 55 kDa that documented as anti-CYP2D6 and anti-uridine triphosphate glucuronosyltransferase autoantibodies when recombinant CYP2D6 and recombinant uridine triphosphate glucuronosyltransferase autoantigens were used for immunoblot, respectively. CONCLUSIONS: A relatively high incidence of anti-liver-kidney microsomal autoantibodies (10.3%) was found in a consecutive sample of Greek patients with hepatitis C. The expanded panel of assays, however, failed to document CYP2D6 as the target autoantigen of anti-liver-kidney microsomal autoantibodies in most patients. We report for the first time the detection of parietal cell antibodies and both anti-CYP2D6 (anti-liver-kidney microsomal type 1) and anti-uridine triphosphate glucuronosyltransferase (anti-liver-kidney microsomal type 3) autoantibodies in patients who were hepatitis C positive/hepatitis D negative. Further studies are needed to confirm our findings and to determine whether these preliminary results have a clinical importance or not.

Confluent hepatic fibrosis as the presenting imaging sign in nonadvanced alcoholic cirrhosis.

Confluent hepatic fibrosis (CHF) is usually a feature of advanced cirrhosis, while occurrence in early stage compensated cirrhosis is uncommon. We report the imaging findings of masslike CHF in a patient with previously unsuspected compensated alcoholic cirrhosis. The combination of partially wedge shape, minimal capsular retraction, and homogeneous delayed enhancement on multiphase contrast-enhanced spiral CT led to the characterization of the lesions and establishment of the diagnosis of cirrhosis.