Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group criteria in acute‐onset autoimmune hepatitis

Background: The diagnosis of autoimmune hepatitis (AIH) is already difficult, and that of acute‐onset AIH with atypical features is even more challenging, even though the revised original diagnostic criteria created by an international AIH group were widely accepted and incorporated into clinical practice. Aims: Recently, simplified diagnostic criteria were proposed. We compared the performance parameters of the simplified scoring system in patients with acute‐onset AIH and examined its usefulness and limitations. Methods: Fifty‐five patients with acute‐onset AIH (29 non‐severe, 14 severe and 12 fulminant) were assessed according to the simplified scoring system and compared with the revised original one. Results: Of the 55 patients, 22 (40%) were diagnosed as ‘definite’ AIH, 28 (51%) as ‘probable’ and five (9%) as ‘non‐diagnostic’ based on the revised original scoring system. By the simplified scoring system, six (11%) were diagnosed as ‘definite’ AIH, 16 (29%) as ‘probable’ and 33 (60%) as ‘non‐diagnostic’. Anti‐nuclear antibody titres did not differ among the three groups. The immunoglobulin G level was higher in fulminant than in non‐severe patients (P=0.01). Sixty‐five per cent showed acute hepatitis (massive necrosis, submassive necrosis and severe acute hepatitis) and 35% showed chronic hepatitis. Conclusions: The revised original scoring system performed better in patients with acute‐onset AIH than the simplified scoring system.     

Diagnostic difficulties,therapeutic strategies,and performance of scoring systems in patients with autoimmune hepatitis and concurrent hepatitis B/C

Abstract

Background. The diagnosis of autoimmune hepatitis (AIH) is already difficult, and that of AIH with chronic viral hepatitis including hepatitis B (HBV) or hepatitis C (HCV) is even more challenging. To date, only a few case-based studies have described this association. Aim. The aim was to retrospectively assess diagnostic difficulties, therapeutic approaches, and performance of the scoring systems in AIH patients with concurrent HBV and HCV. Methods. A total of 25 patients from United States, Sweden, Italy, and Turkey were retrospectively evaluated. Both revised and simplified criteria suggested by the International Autoimmune Hepatitis Group were applied for each patient. All study data were obtained from medical records. Results. Of the 25 patients, 20 (80%) had concomitant HCV and 5 (20%) had HBV. Based on the revised scoring system and simplified criteria, 18 (72%) and 12 (48%) patients were diagnosed as “probable” AIH. None of the patients were diagnosed as “definite” AIH according to both scoring systems. Patients with HCV initially were treated with immunosuppressive agents, and antiviral therapy was commenced when biochemical remission occurred. AIH patients with HBV were first treated with antiviral and thereafter, immunosuppressive therapy was started. Conclusions. This large case series describes concurrent AIH and chronic viral hepatitis. The revised scoring system for AIH had a better performance than the simplified scoring system. However, neither scoring system is optimal for diagnosing AIH alone. In these patients, a definitive diagnosis of AIH should be based on a combination of serological profiles, histological findings, scoring systems, treatment response, and outcomes.     

Re-analysis of clinical features of 89 patients with autoimmune hepatitis using the revised scoring system proposed by the International Autoimmune Hepatitis Group

OBJECTIVE: The diagnostic criteria of autoimmune hepatitis (AIH) were recently modified by the International Autoimmune Hepatitis Group. This study was performed to assess the impact of the revised scoring system on the diagnosis of AIH. PATIENTS AND METHODS: We re-analyzed the clinical features of 89 patients diagnosed as AIH in Nagasaki Prefecture, Japan, using the revised scoring system, and compared the scores and final diagnosis with our previously published results using the original system. RESULTS: Of the 89 patients with AIH, 40 (45%) were classified using the new system as "definite" AIH, 41 (46%) as "probable" AIH, and 8 (9%) patients were categorized as "others". Of these, 37 (42%), 35 (39%), and 4 (4%) patients who were classified as "definite", "probable", and "others" by the original system remained in the same category by the revised system, respectively. However, 3, 4, and 6 patients were re-categorized as "definite" from "probable", "others" from "probable", and "probable" from "definite", respectively. The difference in aggregate scores between the above two systems ranged from -5 to +2. The main contributing factors to the changes in aggregate AIH score were "other autoimmune disease(s)" and "interface hepatitis without lobular involvement and bridging necrosis on liver histology". However, the main contributing factors to the demotions from "definite" to "probable" and form "probable" to "others" were those related to the characteristics of biliary diseases, i.e., antimitochondrial antibody positive, biliary changes in liver histology, and alkaline phosphatase: aspartate aminotransferase ratio between 1.5 and 3.0. Moreover, two patients who had no histological evidence of AIH were both re-categorized as "others" from "probable" AIH. CONCLUSION: Our results indicated that the diagnosis, whether based on the revised or original system, was the same in the majority of AIH patients, but the revised scoring system excluded cases who had features suggestive of biliary diseases from "definite" AIH, and also confirmed that a diagnosis of "definite" AIH should not be made without liver histology.     

Autoimmune acute liver failure: an emerging etiology for intractable acute liver failure

Diagnosis of acute onset autoimmune hepatitis (AIH) is the most challenging task because of atypical clinicopathological features. We examined the nature of acute onset AIH consisting of nonsevere, severe, and fulminant AIH based on our published data and other published papers, and propose how to diagnose and treat this intractable hepatitis. We analyzed clinical, biochemical, immunological, radiological, and histological features of acute onset AIH. Thirty percent of fulminant hepatitis was due to AIH and autoimmune acute liver failure (ALF) was not rare. The important characteristic of acute onset AIH is its histological, radiological, and clinical heterogeneity. Sometimes acute onset AIH develops into ALF in a sub-acute clinical course without appropriate diagnosis and treatment, and becomes resistant to immunosuppressive therapy and has poor prognosis. Unenhanced computed tomography (CT) often shows heterogeneous hypoattenuation in autoimmune ALF. The revised original scoring system (1999) performed better in patients with acute onset AIH than the simplified scoring system (2008). Liver regeneration from periportal progenitor cells to mature hepatocytes was impaired in ALF, resulting in resistance to immunosuppressive therapy. Precise histological evaluation (the presence of centrilobular necrosis/collapse) along with the revised original scoring system and CT findings of heterogeneous hypoattenuation after systematic exclusion of other causes 36 plays an important role in the diagnosis. The most important strategy for autoimmune ALF is to diagnose and treat acute onset AIH before its development into ALF. Liver transplantation should be considered before the occurrence of infectious complications in the case of fulminant liver failure.     

自身免疫性肝炎患者临床病理学特点的性别差异比较

目的 分析自身免疫性肝炎(AIH)患者临床病理学特点是否存在性别差异.方法 169例AIH患者分为男性患者组23例,女性患者组146例,对两组患者的临床资料及肝活组织病理学检查结果进行分析,比较两组患者的临床病理学特点.非正态连续变量的比较采用Mann-Whiteney U检验,分类构成比采用Chi-Squre检验,累积生物化学缓解率采用Kaplan-Meier法.结果 男、女患者组的一般情况、AST、碱性磷酸酶(ALP)、γ-谷氨酰转移酶(GGT)、总胆红素(Tbi)和肝组织学分级比较,差异均无统计学意义(P＞0.05).比较两组患者的血清γ-球蛋白、IgG、IgM、自身抗体阳性率及其伴发其他自身免疫性疾病,差异无统计学意义(P＞0.05).采用传统积分系统评估,男性患者组治疗前积分为14.4±2.3,低于女性患者组的16.6±2.6,两组比较,差异有统计学意义(Z=-3.728,P=0.000),采用简化积分系统评估,男性患者组积分为7.2±0.8,高于女性患者组的6.5±1.2,两组比较,差异有统计学意义(Z=-2.372,P=0.018).15例接受免疫抑制剂治疗的男性AIH患者中有12例达到生物化学缓解,其余3例目前为不完全缓解,累积生物化学缓解率为80％,中位缓解时间为3个月(95％CI为2.070～3.930).结论 AIH在临床及病理特点方面不存在性别间差异,肝功能异常的男性患者也应考虑AIH的可能,建议行肝活组织学检查以明确诊断.简化标准可很好地用于男性AIH患者的临床诊断.     

Overlap of autoimmune hepatitis and primary biliary cirrhosis: an evaluation of a modified scoring system

OBJECTIVE: The coexistence of autoimmune hepatitis (AIH) with primary biliary cirrhosis (PBC) as an overlap syndrome has been previously described. The ability to detect AIH overlap with a revised version of the International Autoimmune Hepatitis Group (IAHG) scoring system, however, remains unknown. Our specific aim was to evaluate the revised IAHG scoring system and its ability to identify AIH overlap in PBC. MErHODS: One hundred forty-one PBC patients with first-time visits to the Mayo Clinic from January 1, 1990 to December 31, 1992 were evaluated. The calculation of individual revised IAHG scores was performed and compared to original IAHG scores. RESULTS: Among 137 PBC patients with available liver histologies, use of the original IAHG scoring system detected "definite" and "probable" AIH overlap among 2.2% and 62% of cases, respectively. Application of the revised IAHG scoring system, however, revealed no individuals (0%) with definite AIH overlap (>15 points). Twenty-six subjects (19%) fulfilled IAHG criteria for probable AIH overlap (10-15 points). The presence of antinuclear antibody and/or smooth muscle antibody positivity (p = 0.05), other autoimmune disorders (p < 0.01), and total histological score (p < 0.001) were significantly greater in the PBC plus probable AIH group than in subjects with PBC alone. CONCLUSION: A reduction in the prevalence of definite 2.2% vs 0%) and probable (62% vs. 19%) AIH overlap among PBC subjects was observed with use of the revised IAHG coring system relative to the original criteria. Applicability of the revised IAHG scoring system, however, remains questionable, as nearly 20% of PBC patients will be classified with probable AIH overlap.     

Evaluation of Validity and Efficiency of Diagnostic Criteria in Autoimmune Hepatitis in Children

Background: Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease with various clinical symptoms, but treatment and prevention of hepatic failure and cirrhosis is possible with early diagnosis. However, no specific test has been approved for the diagnosis of AIH. In 2008, the International Autoimmune Hepatitis Group (IAIHG) developed a simplified diagnostic scoring system that has been widely used in practice. Nevertheless, it cannot distinguish AIH from Primary Sclerosing Cholangitis (PSC) and consensus is lacking with respect to its validity, sensitivity, and applicability for children patients. The newer 2018 version also requires validation. The present study intends to evaluate the validity and efficiency of the IAIHG simplified scoring system and new scoring system in children with AIH.Methods: The present study is a non-interventional case–control study covering 152 patients with hepatic diseases (83 patients with AIH and 69 patients with Wilson disease (WD)). Titers of autoantibodies, IgG levels, hepatic histology, and absence of viral hepatitis were scored and calculated according to IAIHG diagnostic criteria. Statistics software package (SPSS) and draft receiver operating characteristic (ROC) curves was used to analyze data and determine value of diagnostic criteria.Result: In our study, both scoring systems’ accuracy was good in AIH diagnosis, although new score displays higher sensitivity and specificity, suggestive of greater accuracy and predictive strength.Conclusion: Our study is the first validation study of the new scoring system in diagnosing AIH, and further studies require verifying this scoring system.     

Autoimmune hepatitis: East meets west

Autoimmune hepatitis (AIH) is an inflammatory liver disease with diverse clinical spectrum, which predominantly affects females. This review provides detailed comparisons of epidemiology, genetic predispositions, clinical features, risk factors of hepatocellular carcinoma, and mortality in AIH patients between eastern and western countries. AIH prevalence and incidence are lower in Asia‐Pacific area than in Europe and America. European and American patients seem to have more severe disease, characterized with human leukocyte antigen‐DR3 haplotype, younger age, more AIH‐induced “cirrhosis” at diagnosis, higher elevated serum immunoglobulin G levels, and positive rate of antisoluble liver antigen/liver pancreatitis. The overall AIH diagnostic accuracy of revised original criteria and simplified scoring system are similar in European/American populations and Asian. Cirrhosis at presentation and non‐response to immunosuppressive therapy within 1 year are the most important predictors for poor prognosis of AIH patients.     

Clinical features of 89 patients with autoimmune hepatitis in Nagasaki Prefecture, Japan

We examined the clinical characteristics of 89 patients with autoimmune hepatitis (AIH) in Nagasaki Prefecture, Japan, and assessed the usefulness of a provisional scoring system for the diagnosis of AIH proposed by the International Autoimmune Hepatitis Group in 1993. The majority of patients were middle-aged women in their fifties. All patients showed a hepatitic picture. Forty-three patients (48%) had an insidious or chronic onset, while 34 (38%) had an acute onset, and 12 (14%) had liver cirrhosis at presentation. Seventy-nine patients (89%) were positive for antinuclear antibody (ANA), and 5 (6%) were positive for antibody to the hepatitis C virus (anti-HCV). The prognosis was good, with 90% 3-year survival, and most patients responded well to treatment with corticosteroids. The international scoring system was useful for the diagnosis of AIH in most of our patients; the percentages of patients with definite and probable AIH were 48% and 47%, respectively. However, certain factors, such as negative ANA, positive antimitochondrial antibody, concurrent infection with hepatitis B or C virus, and insufficient response to treatment precluded the diagnosis of AIH in some patients. Whether these patients were indeed "true" AIH patients is not clear at present, and further investigation of such patients may be useful for a better understanding of AIH. (Received: June 26, 1998; accepted: Oct. 23, 1998)     

High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis

BACKGROUND/AIMS: Traditionally, autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are regarded as separate disease entities. We report on a group of patients that suggests the existence of an overlap syndrome of the two conditions and on the prevalence of this syndrome among patients with PSC. Furthermore, the impact of the recently revised AIH scoring system for diagnosing AIH in this context was assessed. METHODS: Retrospective analysis of consecutive patients of a tertiary referral centre for liver disease with a diagnosis of PSC. RESULTS: Diagnosis of the overlap syndrome was established for nine patients (8%) of a total group of 113 PSC patients. Four patients initially presented with features of AIH and in five cases PSC was diagnosed first. All patients responded to immunosuppressive therapy; in three cases long-term remission was achieved. Three patients underwent liver transplantation after 4 months and 7 and 9 years, respectively. The original and revised versions of the AIH scoring system gave essentially the same results in the patients with the PSC-AIH overlap syndrome. CONCLUSIONS: Patients with overlapping features of AIH and PSC may be more common than is currently assumed. Recognition of this syndrome is of clinical significance, considering the important therapeutical consequences.     

Primary biliary cirrhosis - Autoimmune hepatitis overlap syndrome: A rationale for corticosteroids use based on a nation-wide retrospective study in Japan

Aims: Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) may simultaneously coexist in some patients, designated as PBC‐AIH overlap syndrome. Previous studies suggest that combination therapy of ursodeoxycholic acid (UDCA) and corticosteroids may be effective. In the current study, we aimed to describe clinical features of these cases and to propose a rationale for combination treatment in PBC‐AIH overlap. Methods: We enrolled patients with PBC‐AIH overlap from eight referral centers for liver diseases in Japan, and clinical, biochemical and immunological features were examined. Liver histology of all patients at diagnosis were analyzed altogether in detail. Eighty‐nine and 44 patients with PBC and AIH alone were included and served as controls. Results: We identified 33 patients with PBC‐AIH overlap. The mean follow‐up period was 6.1 years. On liver histology, the HA (hepatitis activity) score was significantly higher than the CA (cholangitis activity) score (P < 0.001). At the end of the follow‐up period, corticosteroids were used in 23 patients (72%), and neither liver‐related death nor liver transplantation had been noted. The sensitivity and specificity of the simplified AIH scoring system for prediction of patients who required corticosteroids during clinical course was 92% and 75% in the training set (n = 17), and 91% and 80% in the validation set (n = 16) of overlap. Only 3% of PBC patients were diagnosed as having indication for corticosteroid use. Conclusion: In PBC‐AIH overlap, AIH‐like features are dominant in liver histology. The simplified AIH scoring system could predict patients who needed corticosteroids with a higher specificity.     

Applicability of the IAIHG scoring system to the diagnosis of antimitochondrial/anti-M2 seropositive variant form of autoimmune hepatitis

BACKGROUND AND AIMS: According to the International Autoimmune Hepatitis Group (IAIHG) criteria, circulating antimitochondrial antibodies (AMA) do not support the diagnosis of autoimmune hepatitis (AIH). The aims of this study were to characterize a subset of patients with AIH who have AMA and antiM2 seropositivity, and to assess the applicability of the revised scoring system of the IAIHG in the diagnosis of this variant form of AIH. METHODS: Eighteen patients with AMA-AIH were enrolled and compared with 206 classical AIH and 85 primary biliary cirrhosis (PBC) controls. Human leukocyte antigen (HLA) class II alleles were determined by polymerase chain reaction (PCR) amplification with sequence-specific primers, and biopsies were blindly reevaluated. RESULTS: The patients with AMA-AIH were, on average, older than patients with classical AIH and had an hepatocellular pattern of elevated liver enzymes, hypergammaglobulinemia and lower levels of cholesterol, when compared with PBC controls. There were no histological signs of PBC or overlapping forms in any AMA-AIH biopsies. The majority of patients with AMA-AIH carried HLA antigens associated with classical AIH (DRB1*03, n = 5; DRB1*04, n = 7, and DRB1*13, n = 6). Pretreatment scores classified all AMA-AIH patients with probable (n = 17) or improbable (n = 1) AIH. After treatment, only 28% of AMA-AIH patients reached scores for definite diagnosis, compared with 90.1% of AIH-1 and 96.4 AIH-2. In the AMA-AIH group, only patients who relapsed after immunosuppressive drug withdrawal could be classified with definite AIH. CONCLUSIONS: AMA-AIH shares common features with classical AIH. The diagnosis of AMA-AIH may be swayed by the IAIHG criteria, rendering questionable the applicability of the revised scoring system to this variant form of AIH.     

Overlap of autoimmune hepatitis and primary sclerosing cholangitis: an evaluation of a modified scoring system

BACKGROUND/AIM: Recently, the scoring system for the diagnosis of autoimmune hepatitis (AIH) was modified by the International AIH Group. Our aim was to determine the prevalence of AIH in patients with cholangiographically proven primary sclerosing cholangitis (PSC) using this new scoring system. METHODS: A total of 211 PSC patients were evaluated. RESULTS: Three (1.4%) patients scored more than 15 points ('definite' AIH); 13 (6%) patients scored between 10 and 15 points ('probable' AIH); the remaining 195 (93%) patients had less than 10 points, allowing the exclusion of AIH. The separation of patients with PSC plus AIH from patients with PSC alone was based mostly on serum levels of total globulins (p=0.01), IgG (p=0.001), titers of autoantibodies (p<0.001) and histologic score (p<0.001). Using the older scoring system, four (2%) patients met the criteria for the diagnosis of PSC plus 'definite' AIH and 40 (19%) the diagnosis of PSC plus 'probable' AIH. CONCLUSIONS: Overlap of PSC and AIH occurs rarely. The new scoring system seems to more precisely define the potential overlap syndrome between PSC and AIH, although further modification of the new scoring system may provide even better discrimination among these conditions.     

Clinical features of autoimmune hepatitis diagnosed based on simplified criteria of the International Autoimmune Hepatitis Group

BackgroundRecently, simplified diagnostic criteria for autoimmune hepatitis have been proposed.AimWe aimed to evaluate usefulness of the simplified criteria.MethodsWe applied the simplified criteria to 176 autoimmune hepatitis patients diagnosed according to the revised scoring system proposed in 1999 (original criteria). Furthermore, in order to compare predictabilities between these two diagnostic criteria, we included 168 patients with other liver disease than autoimmune hepatitis.ResultsOf 176 autoimmune hepatitis patients, 85% were diagnosed with autoimmune hepatitis according to the simplified criteria, and patients diagnosed according to the simplified criteria showed a higher frequency of antinuclear antibodies and/or smooth muscle antibodies of 1:80 or greater and slightly higher serum levels of immunoglobulin G than those diagnosed according to the original criteria. However, 30% of male patients, 23% of patients with acute presentation, 50% of patients showing histological acute hepatitis and 46% of patients negative for antinuclear antibodies at presentation were not diagnosed with autoimmune hepatitis according to the simplified criteria. The simplified criteria showed lower sensitivity (85% vs. 100%) and higher specificity (99% vs. 93%) for autoimmune hepatitis than the original criteria.ConclusionsThe simplified criteria may be useless for the diagnosis of patients with atypical features, especially patients with histological acute hepatitis.     

Role of international criteria in the diagnosis of autoimmune hepatitis

AIM: To study the clinical and laboratory characteristics of autoimmune hepatitis (AIH), and compare them with International Autoimmune Hepatitis Group (IAHG) criteria. METHODS: Sixty consecutive patients with AIH attended the University Clinic at Tabriz University of Medical Sciences, Iran for a 12mo period and were assessed in a case series study. Serological and biochemical evaluations were carried out in all patients. Autoantibodies, such as antinuclear antibody (ANA), anti-smooth muscle antibody (ASMA), anti-liver-kidney microsomal antibody (ALKM-1) type 1, and perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA) were evaluated in these patients. A liver biopsy was performed after diagnosis of the disease. Patients were evaluated in terms of their signs and symptoms, and laboratory results and the degree to which they corresponded with the diagnostic criteria of IAHG. In this study, both a comprehensive diagnostic scoring system and a simplified diagnostic scoring system were employed for AIH.RESULTS: Sixty patients, 2-0 male, 40 female, mean age 39.45 ± 17.50 years, participated in the study. Treatment began immediately after enrolment into the study. The percent distribution of the study population into definite and probable did not change after the treatment. The most common symptoms in descending order were fatigue (100%), icter (66.7%), abdominal discomfort (33.3%), abdominal distension (2-8.3%), dark urine (2-3.3%), edema (2-3.3%), hematemesis (2-0.0%), pruritus (2-0.0%), melena (11.7%) and pale stool (10.0%). At the physical examination, splenomegaly, ascites, hepatomegaly, epigastric tenderness and an abdominal mass were found in 50.0%, 16.7%, 13.3%, 5.0% and 3.3% of patients, respectively. Hypergammaglobulinemia was detected in 95.0% of cases. ALKM-1, P-ANCA, ANA and ASMA were positive in 71.4%, 66.7%, 42-.4% and 19.4% of cases, respectively. Portal hypertensive gastropathy (45.0%), esophageal varices (41.7%) and cirrhosis (40.0%) were the most prevalent complications of AIH, and there was     

Transitions between variant forms of primary biliary cirrhosis during long-term follow-up

BackgroundConditions exhibiting features of two different autoimmune liver diseases are designated overlap syndromes. Variant forms display some, but not all, characteristics of a distinct autoimmune liver disease. We describe transitions over time between variant forms of PBC, i.e. AMA-negative PBC, autoimmune hepatitis (AIH)–PBC overlap and autoimmune cholangitis (AIC) in a large cohort of PBC patients in Sweden.MethodsWe retrieved all patients with variant forms of PBC in six university hospitals in Sweden, covering 60% of the Swedish population. The diagnosis of PBC and its variants was based on laboratory findings and compatible histological features. The revised autoimmune hepatitis scoring system proposed by the International Autoimmune Hepatitis Group was used to establish the diagnosis of AIH.ResultsIn a population of 800 patients with PBC, we identified 35 (5%) variant forms; 25 patients with AIH–PBC overlap, 8 with AIC and 2 with AMA-negative PBC at the time of our study. The initial diagnoses were PBC (3 patients), AIH (3), AIH–PBC overlap (16), AIC (8) and AMA-negative PBC with (1) or without (4) concomitant AIH. The median follow-up was 125 (41–360) months. Immunosuppression and ursodeoxycholic acid induced a complete or good regression of increased aminotransferases in about half of the patients who were given one or both of these treatments.ConclusionsVariant forms of PBC are seen in approximately 5% of PBC patients in Sweden. Transition between different forms may occur, emphasizing the value of repeat biopsies, but established overlapping AIH–PBC seems to be stable over time.     

Comparison of simplified score with the revised original score for the diagnosis of autoimmune hepatitis: A new or a complementary diagnostic score?

Background and aims

The International Autoimmune Hepatitis Group developed a simplified score for autoimmune hepatitis. We assessed this “new scoring system” and compared it with the International Autoimmune Hepatitis Group original revised score.

Methods

502 patients were evaluated namely, 428 had liver diseases of various etiology [hepatitis B (n = 109), hepatitis C (n = 100), hepatitis D (n = 4), alcoholic liver disease (n = 28), non-alcoholic fatty liver disease (n = 55), autoimmune cholestatic diseases (n = 77), liver disorders of undefined origin (n = 32) and miscellaneous hepatic disorders (n = 23)], 13 had autoimmune hepatitis/overlap syndromes, 18 had autoimmune hepatitis/concurrent with other liver diseases and 43 had autoimmune hepatitis.

Results

The specificity of the simplified score was similar to that of the revised score (97% vs. 97.9%). The sensitivity in unmasking autoimmune hepatitis in autoimmune hepatitis/overlap syndromes was also similar in both systems (53.8% and 61.5%). However, the sensitivity for autoimmune hepatitis diagnosis in autoimmune hepatitis patients with concurrent liver disorders was lower by the new score (p = 0.001). Liver biopsy proved to be the only independent factor for unmasking autoimmune hepatitis component among patients (p = 0.003).

Conclusion

The simplified score is a reliable and simple tool for excluding autoimmune hepatitis. However, both systems cannot unmask autoimmune hepatitis component efficiently in autoimmune hepatitis patients with concurrent autoimmune or non-autoimmune liver diseases. This study also strongly reiterates the importance of liver biopsy in the work-up of patients.     

The international autoimmune hepatitis score in chronic hepatitis C

SUMMARY. Clinical and laboratory findings of autoimmunity are common in chronic hepatitis C. Autoimmune hepatitis (AIH), a disease of unknown cause, has been defined by use of the International Autoimmune Hepatitis Group Score (AIH score), which quantifies clinical and laboratory parameters. To further validate the specificity of the International AIH score and investigate the similarities between hepatitis C and AIH, we measured the International Autoimmune Hepatitis Group Score in patients with well-defined chronic hepatitis C. Thirty consecutive non-cirrhotic patients with chronic hepatitis C were evaluated. Scoring was performed using both components of the AIH score: a set of minimum required parameters including laboratory and historical data and a second set of additional parameters dominated by histological criteria. Autoantibodies were positive in 21 of 30 hepatitis C patients and associated (patient or first-degree relative) autoimmune diseases were present in eight of 30 patients. Histologically, chronic active hepatitis with periportal piecemeal necrosis was seen in 24 of 30 patients and lymphoid follicles in 16 of 30 patients. No patient scored as probable or definite AIH using the minimum required parameters of the AIH score. When histological parameters were included, four of 30 patients scored as probable AIH but none as definite AIH. Therefore, AIH was excluded by the minimal and additional criteria of the AIH score in 86% of patients with hepatitis C despite a high prevalence of autoantibodies in these patients. We conclude that the criteria set forth by the International AIH scoring system defines a distinct disease although it shares some features with chronic hepatitis C. Modification of the AIH scoring system to include other commonly accepted risk factors for hepatitis C and additional histological parameters would further improve its specificity.     

NAFLD and autoimmune hepatitis: Do not judge a book by its cover

Nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease (almost 25% of the general population). Autoimmune hepatitis (AIH) is a relatively rare liver disease of unknown aetiology characterized by female predominance and large heterogeneity regarding epidemiology, clinical manifestations, genetics, serology and liver pathology. The potential NAFLD/AIH coincidence or an AIH diagnosis alone instead of NAFLD represent a challenge for clinicians, both in making a correct and timely diagnosis but also in the management of these diseases. The diagnosis of both diseases can be challenging as: (a) reliable laboratory tests to confidently diagnose or exclude NAFLD or AIH are missing; (b) physicians and pathologists are much more familiar with a very common disease like NAFLD so, they do not consider an alternative or additional diagnosis; (c) most NAFLD studies do not investigate the patients for all autoantibodies involved in AIH diagnosis, apply the diagnostic scoring systems for AIH or address the possibility of AIH features on liver histology and (d) the recent European and American practice guidelines for NAFLD do not mention clearly the importance of IgG determination and liver autoimmune serology according to the AIH guidelines. Patients with NAFLD/AIH coincidence have significantly more frequently hypertension, diabetes, obesity, older age, lower transaminases, bilirubin and simplified score for AIH diagnosis but no female predominance compared to AIH patients only. The true outcome of NAFLD/AIH patients is practically unknown while their management is quite problematic because official clinical practice guidelines for this condition are missing.