Positive Antimitochondrial Antibody Research Articles

Antimitochondrial antibodies of immunoglobulin G3 subclass are associated with a more severe disease course in primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is characterised by the presence of immunoglobulin (Ig) G antimitochondrial antibodies (AMA), which are routinely detected by indirect immunofluorescence (IFL) using composite rodent tissue substrate. The IgG subclass distribution and clinical significance of IFL-detected AMA in patients with PBC have not been previously studied in detail. We have examined IgG subclass-specific AMA detected by IFL on rodent liver, kidney and stomach tissue substrate using affinity-purified IgG subclass monospecific antisera as revealing reagents in 95 AMA-positive PBC patients from Greece. AMA of any of the IgG1, IgG2 or IgG3 subclasses were present in 89/95 (93.7%) patients. Among those 89, 55 (61.8%) had IgG1, 2, 3 AMA positivity; eight (9%) had IgG1, 2; seven (7.9%) had IgG2, 3; eight (9%) had IgG1, 3; nine (10.1%) had IgG1 subclass and two (2.2%) single IgG3 AMA reactivity. IgG4 AMA was absent. IgG3 titres were higher than IgG2 and IgG1 (P<0.001) and IgG1 higher than IgG2 (P<0.001). IgG3 AMA-positive patients had a histologically more advanced disease (P<0.01) and were more frequently cirrhotic compared with those who were negative (P<0.01). There was a positive correlation between AMA IgG3 titre and Mayo risk score (r=0.55, P=0.009, Spearman's correlation). Our findings suggest that AMA are not restricted to a specific IgG subclass. AMA of the IgG3 subclass are associated with a more severe disease course, possibly reflecting the peculiar ability of this isotype to engage mediators of damage.

Incidence, prevalence and clinical course of primary biliary cirrhosis in a Spanish community

Primary biliary cirrhosis (PBC) is characterized by the autoimmune inflammatory response of small intrahepatic bile ducts. Prevalence in Spain is estimated as 61.9 cases per million inhabitants, whereas Northern Europe rates over 200 cases/million. Our objective was to determine the incidence and prevalence of PBC in our health area. PBC was defined by the presence of abnormal liver tests (dissociated cholestasis) with positive antimitochondrial antibodies and/or compatible liver histology. Medical records from patients diagnosed between 1990 and 2002 were reviewed retrospectively. The following data were collected: diagnostic data, demographic and analytic data, liver histology and stage and treatment and disease outcome. In a population of 389 758 inhabitants, 87 patients were diagnosed with PBC. Mean age at diagnosis was 63.9+/-12.6 years. Eighty-four (96.6%) were women. Mean annual incidence was 17.2 per 10 inhabitants and the prevalence at the end of study was 195 per 10. Biopsy was performed in 71 (81.6%) patients, 61 of whom (86%) did not have fibrosis. Time of follow-up was 63.6+/-43.2 (2.28-153.9) months. Incidence and prevalence in our reference area are higher than in some Spanish areas, as per the results previously published; however, they are comparable with those obtained in Northern Europe and the US.

Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disorder that can progress to cirrhosis, shortening life expectancy. PBC patients are often asymptomatic, present with biochemical cholestasis, and test positive (>or=90%) for antimitochondrial antibodies (AMAs) in serum. Although AMA positivity without biochemical cholestasis may indicate increased risk of future PBC development, the contribution of these antibodies to pathogenesis remains enigmatic. Environmental risks and genetic determinants are likely implicated in PBC etiology. Given the familial aggregation of PBC, we hypothesized that AMAs also aggregate among relatives of PBC probands. We investigated the prevalence of AMAs in first-degree relatives (FDRs) of PBC probands to examine whether AMAs aggregate in such pedigrees. Using a PBC family registry, we prospectively screened for AMAs in the serum of 306 FDRs in 145 pedigrees, 350 PBC probands, and 196 controls who were age-matched, sex-matched, race-matched, and residence-matched to probands. The prevalence of AMA in FDRs and controls was 13.1% and 1%, respectively. Greater prevalence of AMA was found in female FDRs of PBC probands [sisters (20.7%), mothers (15.1%), and daughters (9.8%)] than in male FDRs [brothers (7.8%), fathers (3.7%), and sons (0%)]. AMAs aggregate among FDRs of PBC probands. Our data have clinical implications for FDRs of PBC probands because AMA positivity may suggest susceptibility to PBC. Thus, the identification and follow-up of these relatives may lead to earlier disease diagnosis and treatment. Furthermore, if AMA development is heritable, this trait will provide a basis to dissect the genetic predisposition to PBC.

Prevalence and Predictors of Esophageal Varices in Patients With Primary Biliary Cirrhosis

Esophageal varices and bleeding are among the most feared complications of primary biliary cirrhosis (PBC). We aimed to determine the prevalence of esophageal varices in patients with PBC, to evaluate noninvasive markers of esophageal varices in this population, and to validate the results in an independent set of patients. Data were collected on all patients with PBC seen for the first time at the University of Florida (study group) and at Case Western Reserve University hospitals (cross-validation group) during 7 consecutive years. Logistic regression analysis was used to identify independent predictors of esophageal varices. The best cut-off values were calculated based on receiver operating characteristic curves. The diagnostic accuracy of the independent predictors of esophageal varices identified in the study group were retested in the cross-validation group. Of 210 patients with PBC seen at the University of Florida, 113 had an endoscopy and 49.6% (56 of 113) were found to have esophageal varices. After excluding 22 patients with a history of variceal bleeding, data on 91 patients were analyzed. Thirty-four patients had esophageal varices (37%). Multivariate analysis revealed that a platelet count of less than 140,000 (odds ratio, 7.6; 95% confidence interval, 1.6-37) and a Mayo risk score of 4.5 or greater (odds ratio, 10.6; 95% confidence interval, 1.8-62) were independent predictors of esophageal varices. The diagnostic accuracy of these predictors was confirmed in an independent set of patients. Among patients with PBC, a platelet count of less than 140,000 and/or a Mayo risk score of 4.5 or greater appears to identify those patients more likely to benefit from a screening endoscopy.

Clinical Epidemiology of Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology. It is typically characterized by a trio of criteria including cholestatic liver biochemistry tests for at least 6 month's duration, the presence of a positive serum antimitochondrial antibody, and histologic findings from liver biopsy that are compatible or diagnostic with PBC. Although no unifying mechanism of action has been described to explain the pathogenesis of PBC to date, the natural history of this condition is greatly influenced by several known and unknown risk factors. In turn, a number of publications have focused on describing the burden of disease and prognosis in patients with PBC, This review article will summarize the descriptive epidemiology of PBC including new insights on risk factors, prognosis, and the effect of medical therapy in contemporary patients.

Prevalence and incidence of primary biliary cirrhosis are increasing in Finland

Objective. To examine the epidemiology of primary biliary cirrhosis (PBC) in Finland and to evaluate whether the possible increase in prevalence was attributable to the increasing incidence, better survival, or both. Material and methods. The Hospital Discharge Register, pathology registers, and death certificates for the years 1988–99 were scrutinized, and the patients identified were followed-up for survival until 31 October 2004. The study area covered four university hospital districts: a total of 25 hospitals. The diagnosis of PBC was regarded as definite (or probable) if three (or two) of the following criteria were fulfilled: positive antimitochondrial antibodies, constantly elevated alkaline phosphatase, and compatible liver histology. Results. In the total population of the study areas, the age-standardized prevalence of PBC increased during the study period from 103 (95% CI: 97–110) to 180 (172–189) per million inhabitants. Incidence increased from 12 (10–14) to 17 (15–20) per million inhabitants per year. The annual average increase in prevalence was 5.1% (4.2–5.9%, p<0.0001) and in incidence 3.5% (0.9%–6.0%, p=0.008). In gender-specific analyses among women, the prevalence of PBC increased from 161 (151–171) to 292 (277–207) per million during the study period and the incidence from 20 (16–24) to 27 (23–32) per million per year. The death rate was 4% per year and half the deaths were from liver-related causes. Survival after diagnosis during the study period lengthened. Conclusions. The prevalence of PBC increased in Finland during 1988–99, owing to both the increased incidence and the prolonged survival.

Differences between Caucasian, African American, and Hispanic patients with primary biliary cirrhosis in the United States

Primary biliary cirrhosis (PBC) is an uncommon chronic cholestatic liver disease that primarily afflicts young and middle-aged Caucasian women; there are limited data on the clinical presentation and disease severity among non-Caucasian patients with this disease. The goal of this study was to examine differences in the severity of liver disease between Caucasian and non-Caucasian patients with PBC screened for enrollment in a large national multicenter clinical trial. Demographic features, symptoms, physical findings, and laboratory tests obtained during screening were examined in 535 patients with PBC with respect to ethnicity, gender, and antimitochondrial antibody (AMA) status; 73 of 535 (13.6%) were non-Caucasian (21 were African American, and 42 were Hispanic). Non-Caucasians were more likely than Caucasians to be ineligible for participation in the clinical trial (46.5% versus 25.1%, P = 0.0001), primarily because of greater disease severity. African Americans and Hispanics were also more likely to have a lower activity level, more severe pruritus, and more advanced disease. However, the mean age, male-to-female ratio, and seroprevalence of AMA positivity were similar between the 2 groups. Liver disease severity at clinical presentation is higher among non-Caucasians than Caucasians with PBC, and this cannot be explained by demographic or serologic features alone. Possible mechanisms underlying this health discrepancy are not clear, but increased awareness of PBC as a cause of chronic cholestatic liver disease is critical in evaluating non-Caucasian patients in the United States.

Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia

Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments.

Primary biliary cirrhosis (PBC) with positive anti-mitochondrial antibody (AMA) and negative M2 subfraction – a cautionary tale

Introduction: PBC is almost invariably associated with M2-AMA positivity. We report a series of 4 patients who presented with a PBC-like syndrome, with positive AMA and invariably elevated IgM, in whom the M2 sub-fraction was negative by immunoblot technique. Subsequent external testing for M2 by ELISA was positive

Serum Reactivity Against Bacterial Pyruvate Dehydrogenase: Increasing the Specificity of Anti‐Mitochondrial Antibodies for the Diagnosis of Primary Biliary Cirrhosis

Antimitochondrial antibodies (AMA) are the serum hallmark of primary biliary cirrhosis (PBC). However, AMA-positivity can be found in non-PBC sera when lower dilutions are used, thus raising issues about the specificity and sensitivity of the test. AMA reacts primarily with the lipoylated domains of pyruvate dehydrogenase-E2 (PDC-E2) which is highly conserved across species, including bacteria. We studied 77 serum samples, including 24 from patients with anti-PDC-E2-positive PBC and 53 controls (16 with autoimmune hepatitis (AIH), 10 with primary sclerosing cholangitis (PSC), and 27 healthy individuals) for their reactivities at serial dilutions (1:10, 1:20 and 1:40) against Escherichia coli DH5 alpha lysate overexpressing human PDC-E2 using immunoblotting (IB). A murine anti-human PDC-E2 monoclonal antibody (mAB) was used as control. We further studied positive sera using adsorption with a synthetic E. coli peptide sharing similarity with human PDC-E2. Finally, we verified whether a unique buffer for E. coli preparation could reduce non-specific serum reactivity. Results demonstrated that 100% of anti-PDC-E2-positive PBC and up to 38% of control sera at 1:10 dilution recognized E. coli PDC-E2 at IB while dilution tests indicated that the overall potency of PBC reactivity was 100-fold higher compared to controls. In fact, a subgroup (20-38%) of non-PBC sera were positive at low titers but lost the reactivity when absorbed with the synthetic E. coli peptide. Finally, our unique buffer reduced the reactivity of non-PBC sera as measured by ELISA. In conclusion, we demonstrated that weak cross-reactivity with E. coli PDC-E2 occurs in non-PBC sera at lower dilutions and that such reactivity is not due to AMA-positivity. The use of a specific buffer might avoid the risk of false positive AMA determinations when E. coli-expressed recombinant antigens are used.

Antimitochondrial antibodies in patients with chronic hepatitis C virus infection: description of 18 cases and review of the literature

To describe the clinical and immunologic patterns of disease expression of patients with chronic hepatitis C virus (HCV) infection and positive antimitochondrial antibodies (AMA). We investigated the presence of AMA in 237 consecutive HCV patients with extrahepatic manifestations from an International Registry. AMA were detected by indirect immunofluorescence in triple rat tissue (liver, stomach and kidney), aceton-fixed criosections and FITC-conjugated rabbit anti-human immunoglobulins. We found positive AMA in 18 (8%) out of 237 HCV patients. All patients were female with a mean age at protocol inclusion of 65.8 years (ranging from 37 to 87 years). Twelve (67%) patients fulfilled classification criteria for systemic autoimmune diseases (SAD), including Sjögren's syndrome (n = 7), systemic sclerosis (n = 3) and systemic lupus erythematosus (n = 2). Fourteen (78%) of the HCV-AMA patients presented at least one of the highly suggestive characteristics of primary biliary cirrhosis (PBC): 9 (50%) had a specific M2 pattern, 6 (33%) had more than twice normal levels of alkaline phosphatase, 5 (28%) had raised IgM levels and 4 (22%) a histological pattern compatible with PBC. Five (28%) patients developed neoplasia after detection of AMA. Seven (39%) patients died, due to neoplasia (n = 4), cirrhotic complications (n = 2) and hepatopulmonary syndrome (n = 1). We describe a subset of HCV patients with positive AMA who presented a broad spectrum of clinical features, including liver, autoimmune and neoplasic manifestations. Two-thirds of these patients presented an associated SAD, mainly Sjögren's syndrome or systemic sclerosis, together with a high frequency of multiple autoantibodies and an increased prevalence of cirrhosis and neoplasia.

Oncocytic carcinoma in the submandibular gland: Report of a case based on anti-mitochondrial immunohistochemical observations

Oncocytic carcinoma arising in the submandibular gland is a very rare tumor that has only previously been reported in nine cases. This paper describes an additional case of oncocytic carcinoma in the right submandibular gland. The patient was a 55-year-old Japanese male who was presented with a 3 cm right submandibular mass. Both radical resection of the tumor and right supraomohyoid neck dissection were performed. Histologically, the tumor cells exhibited an abundant eosinophilic cytoplasm, which appeared to be finely granular, and invaded the surrounding tissues. In addition, perineural invasion was also observed. Electron microscopy demonstrated the presence of an abundance mitochondria in the cytoplasm of the tumor cells. However, since the fine structure of cytoplasm was destroyed by fixation and embedding of the tissue for the light microscopy, the cytoplasm of the tumor could not be observed clearly. Immunohistochemically, anti-mitochondrial antibody positivity was evident in the cytoplasm of the tumor cells. Consequently, we diagnosed the mass as oncocytic carcinoma. Usually, electron microscopy is necessary to diagnose oncocytic lesions, but the paraffin-embedded tissue was not suitable for electron microscopy. Immunohistochemistry using an anti-mitochondrial antibody was found to be useful and helpful for the diagnosis of oncocytic lesions.

The natural history of asymptomatic primary biliary cirrhosis

Appreciation of the spectrum of clinical features of primary biliary cirrhosis (PBC) has evolved considerably over the past few decades. Although the substantial female predominance documented in early series remains true today, it has become clear that other so called ‘classical’ clinical features, such as symptoms of jaundice, pruritis and fatigue, and even biochemical markers of cholestasis, need not necessarily be present. Only a small minority of patients nowadays present with liver failure and most are even identified while asymptomatic, with up to 80% of patients in recently reported series having no PBC-related symptoms at the time of diagnosis, compared to only 4% some 30 years ago (Table 1).1–7 The diagnosis of PBC in asymptomatic patients is usually established after the chance finding of an abnormal physical sign, such as hepatomegaly, or, more often, an elevated serum alkaline phosphatase level during the course of an unrelated illness. Alternatively, patients may be diagnosed after biochemical or autoantibody, including anti-mitochondrial antibody (AMA), screening is carried out either as part of a routine health check or following the documentation of one of the many extrahepatic, predominantly autoimmune, disorders known to be associated with PBC. Crucial to the validity of diagnosing PBC on the basis of a positive AMA test in the latter circumstance is the finding that over 93% of AMA-positive yet asymptomatic patients with no biochemical evidence of cholestasis nonetheless have histological changes compatible with (or diagnostic of) PBC at liver biopsy.8 View this table: Table 1 Prevalence of symptoms at diagnosis of PBC Recognition of the extended clinical spectrum of PBC has resulted in a substantial increase in its reported prevalence, at least in some areas. Far from the historical view of PBC as an uncommon condition,9 recent studies from the north-east of England and Olmstead County in the US indicate that … Address correspondence to A/Professor S.M. Riordan, Gastrointestinal and Liver Unit, The Prince of Wales Hospital, Barker Street, Randwick 2031, New South Wales, Australia. email: riordans{at}sesahs.nsw.gov.au

Does the diagnosis of primary biliary cirrhosis or autoimmune cholangitis depend on the ‘phase’ of the disease?

It is unclear whether autoimmune cholangitis (AIC) is a separate disease entity or primary biliary cirrhosis (PBC) without antimitochondrial antibodies (AMA) since fluctuation of AMA titres by immunofluorescence (IF) is often observed during the course of PBC. The aim of this study was to determine the serial changes in AMA profiles during the course of initially diagnosed PBC or AIC. In this prospective study, 32 patients with PBC or AIC were followed-up for at least 20 months and tested for AMA by IF, enzyme-linked immunosorbent assay (ELISA), and immunoblotting (IB). When positive AMA result was defined as 'AMA by IF positive', 'AMA by IF and/or ELISA positive', and 'AMA by IB positive', the diagnosis of PBC or AIC did not change in 78%, 91%, and 97%, respectively, throughout follow-up. However, the diagnosis changed in one patient, and three patients were diagnosed as AIC throughout follow-up, despite the use of all three assays. Our results suggested that the diagnosis of PBC and AIC was dependent on the 'phase' of the respective disease in 22% of the patients when negative AMA result was defined as 'AMA by IF negative'. This may result in recommending IB analysis before making the diagnosis of AIC.

Rapid-onset Primary Biliary Cirrhosis Resembling Drug-induced Liver Injury

A 54-year-old woman was admitted to our hospital because of acute liver injury. Since she had a history of having used a diet product, drug-induced liver injury (DILI) was initially considered. However, the patient was subsequently diagnosed as suffering from primary biliary cirrhosis (PBC) based on the findings of liver histology and serum anti-mitochondrial antibody positivity. Overlap syndrome between PBC and autoimmune hepatitis (AIH) was also suspected, however, serum levels of aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase became normal three months later, after treatment with combination therapy comprising ursodeoxycholic acid plus bezafibrate. We therefore concluded that the liver disease in this patient was actually PBC, but that it resembled overlap syndrome or DILI. In cases of PBC, a rapid onset, as frequently seen in the case of DILI, viral hepatitis or AIH, is not common. We herein report a rare case of PBC which resembled DILI.

Case Report: Histologic Features of Steatohepatitis in Patients with a Clinical Diagnosis of Autoimmune Cholestasis

Nonalcoholic steatohepatitis (NASH) is a disease included in the spectrum of nonalcoholic fatty liver disease, which is able to continue progression toward cirrhosis (1). The definition of NASH relies on the common exclusion of significant alcohol use (<20 g/day) and the characteristic histological lesions include some degree of steatosis in addition to mixed lobular inflammation and hepatocyte damage (ballooning degeneration with or without Mallory’s hyaline) (2). NASH has been found in patients suffering from chronic hepatitis C (3) and also in other forms of liver diseases, such as chronic hepatitis B, autoimmune hepatitis, drug-induced liver disease, and α-1 antitrypsin deficiency (4). Primary biliary cirrhosis (PBC) should be considered when patients show a biochemical and histological cholestatic pattern along with positive serum antimitochondrial antibodies (AMA), including immunoblot studies, or PBC-specific antinuclear antibodies (ANA), mainly anti-SP100 and anti-GP210 (5), while patients showing a cholestatic pattern together with negative AMA and positive ANA (different from rim-like or multiple nuclear dots) need to be classified as having autoimmune cholangitis (AIC) (5, 6). Differences found between both groups according to biochemical or histological features are minimal. Indeed, histopathologic features of both entities consist of characteristic interlobular and septal bile ducts lesions (florid duct lesion), cholestasis, ductular proliferation, portal and periportal hepatitis, usually mild

When is liver biopsy needed in the diagnosis of primary biliary cirrhosis?

Background & Aims: Liver biopsy has been largely replaced by less invasive measurements applied to mathematical models in defining the prognosis of patients with primary biliary cirrhosis (PBC). The need for liver biopsy in the diagnosis of patients with positive antimitochondrial antibodies (AMAs) in the setting of biochemical cholestasis remains uncertain. This study was undertaken to determine which variables indicate the need for liver biopsy in patients with positive AMA and suspected PBC. Methods: A total of 3198 patients that were tested for AMA in 1999 and 2000 were identified. Of these, 198 (6.2%) were AMA positive. Forty-two patients were excluded because of unavailable laboratory data or liver biopsy specimens. Data on the remaining 156 patients were analyzed to determine which variables among patients with positive AMA with and without a histopathologic diagnosis of PBC indicate the need for liver biopsy. Results: In 131 of 156 (84.6%) patients, a diagnosis of PBC was established by liver biopsy. The histological diagnosis of PBC was associated with a cholestatic biochemical profile (alkaline phosphatase [AP] >1.5 times the upper limits of normal [ULN] and aspartate aminotransferase [AST] <5 times ULN) in 112 of 131 (85.5%) patients. The combination of AP >1.5 times the ULN and AST <5 times the ULN yielded a 98.2% positive predictive value of PBC diagnosis on liver biopsy in AMA-positive subjects. These results were corroborated by cross-validation in an independent set of patients. Conclusions: In patients with positive AMA, a cholestatic biochemical profile (AP >1.5 times the ULN), and absence of markedly elevated AST (<5 times the ULN), liver biopsy is rarely required to establish the diagnosis of PBC. Liver biopsy would be beneficial to establish the diagnosis of PBC in only a minority of AMA-positive patients with AP <1.5 times the ULN or AST >5 times the ULN.

Deranged liver function tests in type 1 diabetes mellitus: an unusual presentation of Treponema pallidum infection

Deranged liver function tests are common in the diabetic population. Acute derangements are less common, however, and are usually secondary to viral or drug-related causes. A case of syphilitic hepatitis associated with positive anti-mitochondrial antibodies is described, where diagnostic confusion was present until characteristic features of the secondary phase of syphilis infection occurred.

Prevalence of autoimmune liver disease in Alaska Natives.

There is limited information on the prevalence of autoimmune liver disease in nonwhite populations. We conducted a population-based study on the prevalence of autoimmune liver diseases in Alaska natives. Clinical records from 1984 to July, 2000 were reviewed to identify Alaska natives with autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis, autoimmune cholangitis, and overlap syndromes of two of the above. AIH was defined as definite or probable, based on criteria established by the International Autoimmune Hepatitis Group. The diagnosis of PBC was based on a positive antimitochondrial antibody of > or = 1: 40, biochemical evidence of cholestasis, and compatible liver biopsy. Autoimmune cholangitis was defined as PBC but without a positive antimitochondrial antibody. Primary sclerosing cholangitis was diagnosed on the basis of cholangiogram. Seventy-seven patients with possible autoimmune liver disease were identified. Of these, 42 had definite and seven probable AIH. At presentation, 34.7% of patients with AIH presented with acute icteric hepatitis, and 65.3% were asymptomatic. Persons presenting with mild or no symptoms were more likely to have moderate to severe fibrosis on liver biopsy than those presenting with jaundice. Eighteen persons were diagnosed with PBC, five with autoimmune cholangitis, five with overlap syndrome, and none with primary sclerosing cholangitis. The combined point prevalence of AIH Alaska natives was 42.9/100,000 (95% CI = 31-57.7). The prevalence of PBC was 16/100,000 (95% CI = 12.9-25.4). This population-based study demonstrates that the prevalence rates of AIH and PBC in Alaska natives are comparable with reported rates in other populations.

Prevalence of autoimmune liver disease in Alaska natives

OBJECTIVE: There is limited information on the prevalence of autoimmune liver disease in nonwhite populations. We conducted a population-based study on the prevalence of autoimmune liver diseases in Alaska natives. METHODS: Clinical records from 1984 to July, 2000 were reviewed to identify Alaska natives with autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis, autoimmune cholangitis, and overlap syndromes of two of the above. AIH was defined as definite or probable, based on criteria established by the International Autoimmune Hepatitis Group. The diagnosis of PBC was based on a positive antimitochondrial antibody of ≥1:40, biochemical evidence of cholestasis, and compatible liver biopsy. Autoimmune cholangitis was defined as PBC but without a positive antimitochondrial antibody. Primary sclerosing cholangitis was diagnosed on the basis of cholangiogram. RESULTS: Seventy-seven patients with possible autoimmune liver disease were identified. Of these, 42 had definite and seven probable AIH. At presentation, 34.7% of patients with AIH presented with acute icteric hepatitis, and 65.3% were asymptomatic. Persons presenting with mild or no symptoms were more likely to have moderate to severe fibrosis on liver biopsy than those presenting with jaundice. Eighteen persons were diagnosed with PBC, five with autoimmune cholangitis, five with overlap syndrome, and none with primary sclerosing cholangitis. The combined point prevalence of AIH Alaska natives was 42.9/100,000 (95% CI = 31–57.7). The prevalence of PBC was 16/100,000 (95% CI = 12.9–25.4). CONCLUSIONS: This population-based study demonstrates that the prevalence rates of AIH and PBC in Alaska natives are comparable with reported rates in other populations.