• Antiphospholipid syndrome
• Extra-criteria antiphospholipid antibodies
• IgA anti-β2 glycoprotein
• IgG anti-phosphatidylserine/prothrombin
• Small vessel brain lesions

• anti-beta 2-glycoprotein I antibodies
• antiphospholipid antibodies
• arthralgia
• arthritis

• Male
• Female
• Humans
• Adult
• Middle Aged
• Antiphospholipid Syndrome
• Antibodies, Antiphospholipid
• Antibodies, Anticardiolipin
• Autoantibodies
• beta 2-Glycoprotein I
• Arthritis/epidemiology
• Immunoglobulin A
• Arthralgia

• Antimitochondrial antibodies
• Autoantibodies
• Biliary liver disease
• Cholangitis
• Diagnosis

The specific value of IgA Anti-β2glycoprotein I antibodies (aB2GP1) in the diagnosis and management of antiphospholipid syndrome (APS) is still controversial and a matter of active debate. The relevance of the IgA aB2GP1 isotype in the pathophysiology of APS has been increasingly studied in the last years. There is well know that subjects with multiple positive APS tests are at increased risk of thrombosis and/or miscarriage. However, these antibodies are not included in the 2006 APS classification criteria. Since 2010 the task force of the Galveston International Congress on APS recommends testing IgA aB2GP1 isotype in patients with APS clinical criteria in the absence of criteria antibodies. In this review, we summarize the molecular and clinical “state of the art” of the IgA aB2GP in the context of APS. We also discuss some of the characteristics that may help to evaluate the real value of the IgA aB2GP1 determination in basic research and clinical practice. The scientific community should be aware of the importance of clarifying the role of IgA aB2GP1 in the APS diagnosis.

• antiphospholipid antibodies
• antiphospholipid syndrome
• domain of Beta 2 Glycoprotein I antibodies
• immunology
• non-criteria
• recurrent pregnancy loss
• stroke
• thrombosis

This retrospective study at a single center aimed to evaluate the role of the red blood cell distribution width (RDW)-to-platelet ratio and other laboratory indices associated with the severity of histological hepatic fibrosis on liver biopsy in patients with autoimmune hepatitis (AIH).

We retrospectively reviewed records from 2097 adult patients who had liver biopsies. Of these patients, data from 72 with AIH and 164 with drug-induced liver injury (DILI) with complete laboratory information and medical histories were included in the analysis.

We found that compared with patients with DILI, patients with AIH had higher alkaline phosphatase, globulin, and total bile acid levels. Multivariate analyses of risk factors for AIH-associated advanced liver fibrosis in Chinese patients revealed an estimated adjusted odds ratio (AOR) (95% CI) of 1.609 (1.028–2.517) in patients with higher immunoglobulin A (IgA) levels. Patients with higher gamma-glutamyl transpeptidase (GGT)-to-platelet ratio (GPR) values had a significantly higher risk of serious liver fibrosis than patients with lower GPR values. Advanced fibrosis risk was higher in patients with higher RPR values than in patients with lower RPR values [AOR (95% CI): 25.507 (2.934–221.784)]. The result for area under the curve (0.821) analysis for lnRPR levels indicated this variable had high diagnostic performance for predicting advanced AIH-related fibrosis.

The degree of histological liver fibrosis in patients with AIH was significantly associated with an increased red blood cell distribution width-to-platelet ratio, GPR, and increased serum levels of IgA.

• Anti-β2GPI
• Autoimmunity
• Rheumatological disorder
• Thrombosis

• Antiphospholipid syndrome
• anticardiolipin antibodies
• infection
• meta-analysis
• observational studies
• systematic review
• thromboembolic events

• Autoantibodies
• Geoepidemiology
• Liver autoimmunity
• Natural history
• Primary biliary cholangitis
• Primary biliary cirrhosis
• Prognosis

• Adult
• Aged
• Alcohol Drinking/epidemiology
• Autoantibodies/blood
• Carcinoma, Hepatocellular/epidemiology
• Cholangitis/complications
• Cholangitis/therapy
• Female
• Greece/epidemiology
• Humans
• Liver Cirrhosis, Biliary/epidemiology
• Liver Neoplasms/epidemiology
• Liver Transplantation
• Logistic Models
• Male
• Middle Aged
• Multivariate Analysis
• Retrospective Studies
• Risk Factors
• Seasons
• Survival Analysis
• Treatment Outcome

• APRI
• Cartilage oligomeric matrix protein
• Cirrhosis
• Hepatitis B
• Hepatitis C
• Liver fibrosis

• Adult
• Aged
• Aspartate Aminotransferases/blood
• Biomarkers/blood
• Cartilage Oligomeric Matrix Protein/blood
• Case-Control Studies
• Disease Progression
• Elasticity Imaging Techniques
• Female
• Greece
• Hepatitis B, Chronic/complications
• Hepatitis C, Chronic/complications
• Humans
• Liver/pathology
• Liver Cirrhosis/blood
• Male
• Middle Aged
• Platelet Count
• ROC Curve
• Sensitivity and Specificity
• Severity of Illness Index
• Young Adult

Antiphospholipid syndrome (APS) is a thrombophilic disorder that classically presents with vascular thrombosis and/or obstetric complications. APS is associated with antiphospholipid antibodies: a heterogeneous group of autoantibodies that are directed against membrane phospholipids in complex with phospholipid-binding proteins. Beta-2-glycoprotein I (B2GPI) binds anionic phospholipids and is considered to be the predominant antigen in APS and antibodies against B2GPI (anti-B2GPI) are recognised in the laboratory criteria for APS diagnosis. This review focuses on the part played by anti-B2GPI in the pathogenesis of APS, their associations with different clinical phenotypes of the disorder and new avenues for refining the diagnostic potential of anti-B2GPI testing.

• antiphospholipid antibody
• antiphospholipid syndrome
• beta-2 glycoprotein I
• domain specific antibodies
• isotypes

• Autoantibody
• Biliary tree
• Cholestatic diseases
• Laboratory tools
• Mitochondria
• Nuclear proteins

• Antibodies, Antinuclear/blood
• Autoantibodies/blood
• Autoantigens/immunology
• Autoimmune Diseases/diagnosis
• Autoimmune Diseases/immunology
• Cholangitis, Sclerosing/diagnosis
• Cholangitis, Sclerosing/immunology
• Dihydrolipoyllysine-Residue Acetyltransferase/immunology
• Enzyme-Linked Immunosorbent Assay
• Fluorescent Antibody Technique, Indirect
• Humans
• Liver Cirrhosis, Biliary/diagnosis
• Liver Cirrhosis, Biliary/immunology
• Mitochondrial Proteins/immunology

• IgA anti-β2 glycoprotein I
• Systemic lupus erythematosus
• antiphospholipid antibodies
• antiphospholipid syndrome

• Anti-cardiolipin antibodies
• Anti-phosphatidylserine/prothrombin
• Anti-β2-Glycoprotein-I antibodies
• Antiphospholipid antibodies
• Crohn’s disease
• Disease progression
• Thrombosis
• Ulcerative colitis

• Adult
• Antibodies, Antiphospholipid/blood
• Biomarkers/blood
• Colitis, Ulcerative/blood
• Colitis, Ulcerative/diagnosis
• Colitis, Ulcerative/epidemiology
• Colitis, Ulcerative/immunology
• Crohn Disease/blood
• Crohn Disease/diagnosis
• Crohn Disease/epidemiology
• Crohn Disease/immunology
• Crohn Disease/therapy
• Disease Progression
• Female
• Humans
• Hungary/epidemiology
• Male
• Middle Aged
• Phenotype
• Predictive Value of Tests
• Prevalence
• Prospective Studies
• Risk Factors
• Seroepidemiologic Studies
• Serologic Tests
• Tertiary Care Centers
• Young Adult

• Anti-β2 glycoprotein-I antibodies
• Anticardiolipin antibodies
• Antiphospholipid antibodies
• Viral hepatitis

Autoimmune hepatitis (AIH) is an unresolving progressive liver disease of unknown etiology characterized by hypergammaglobulinemia, autoantibodies detection and interface hepatitis. Due to the absence of specific diagnostic markers and the large heterogeneity of its clinical, laboratory and histological features, AIH diagnosis may be potentially difficult. Therefore, in this in-depth review we summarize the substantial progress on etiopathogenesis, clinical, serological and histological phenotypes of AIH. AIH has a global distribution affecting any age, both sexes and all ethnic groups. Clinical manifestations vary from asymptomatic to severe or rarely fulminant hepatitis. Hypergammaglobulinemia with selective elevation of IgG is found in most cases. Autoimmune attack is perpetuated, possibly via molecular mimicry, and favored by the impaired control of T-regulatory cells. Histology (interface hepatitis, emperipolesis and hepatic rosette formation) and autoantibodies detection although not pathognomonic, are still the hallmark for a timely diagnosis. AIH remains a major diagnostic challenge. AIH should be considered in every case in the absence of viral, metabolic, genetic and toxic etiology of chronic or acute hepatitis. Laboratory personnel, hepato-pathologists and clinicians need to become more familiar with disease expressions and the interpretation of liver histology and autoimmune serology to derive maximum benefit for the patient.

• Autoimmune hepatitis
• Liver autoimmunity
• Liver-related autoantibodies
• Non-organ specific autoantibodies
• Overlap syndromes

• Animals
• Autoantibodies/blood
• Biomarkers/blood
• Hepatitis, Autoimmune/blood
• Hepatitis, Autoimmune/classification
• Hepatitis, Autoimmune/diagnosis
• Hepatitis, Autoimmune/epidemiology
• Hepatitis, Autoimmune/immunology
• Hepatitis, Autoimmune/therapy
• Humans
• Liver/immunology
• Liver/pathology
• Phenotype
• Prognosis
• Risk Factors

• Biological Markers
• Fibrosis
• Immunoglobulin A
• Liver Cirrhosis
• Non-alcoholic Fatty Liver Disease

• Adult
• Area Under Curve
• Biomarkers/blood
• Biopsy
• Case-Control Studies
• Disease Progression
• Female
• Humans
• Immunoglobulin A/blood
• Liver Cirrhosis/blood
• Liver Cirrhosis/diagnosis
• Liver Cirrhosis/etiology
• Liver Cirrhosis/immunology
• Male
• Middle Aged
• Non-alcoholic Fatty Liver Disease/blood
• Non-alcoholic Fatty Liver Disease/complications
• Non-alcoholic Fatty Liver Disease/diagnosis
• Non-alcoholic Fatty Liver Disease/immunology
• Predictive Value of Tests
• Prognosis
• ROC Curve
• Reproducibility of Results
• Serologic Tests
• Severity of Illness Index
• Up-Regulation

• Tunisia
• anti-beta 2 glycoprotein I antibodies
• anticardiolipin antibodies
• antiphospholipid antibodies
• primary biliary cirrhosis

• Anti-gp210 antibodies
• Anti-mitochondrial antibodies
• Anticentromere antibodies
• Autoantibodies
• Primary biliary cirrhosis

• Animals
• Antibodies, Antinuclear/blood
• Apoptosis
• Autoantibodies/blood
• Autoimmunity
• Bile Ducts, Intrahepatic/immunology
• Bile Ducts, Intrahepatic/pathology
• Biomarkers/blood
• Humans
• Liver Cirrhosis, Biliary/blood
• Liver Cirrhosis, Biliary/immunology
• Liver Cirrhosis, Biliary/pathology
• Mitochondria/immunology
• Prognosis

• Aged
• Antibodies, Antinuclear/biosynthesis
• Autoantibodies/biosynthesis
• Disease Progression
• Female
• Follow-Up Studies
• Humans
• Immunoglobulin A/biosynthesis
• Immunoglobulin G/biosynthesis
• Immunoglobulin Isotypes/biosynthesis
• Liver Cirrhosis, Biliary/immunology
• Liver Cirrhosis, Biliary/metabolism
• Liver Cirrhosis, Biliary/pathology
• Male
• Middle Aged
• Mitochondria/immunology

AIM: To present the characteristics, management and outcome of patients with hepatitis B virus (HBV) or hepatitis C virus (HCV) infections concurrent with primary biliary cirrhosis (PBC).

METHODS: Since January 2001 to September 2009, we retrospectively evaluated the medical records of all HBV (n = 1493) and HCV patients (n = 526) who are followed in our center for the presence of concurrent PBC. Seventeen patients identified with concurrent viral hepatitis and PBC (8 HCV and PBC; follow-up: 61 ± 37 mo and 9 HBV and PBC; follow-up: 57 ± 38 mo). PBC diagnosis was established if the patients met at least two of the following criteria: positivity for antimitochondrial antibody, elevated cholestatic enzymes and histological lesions of PBC.

RESULTS: HCV or HBV diagnosis preceded that of PBC in most patients by many years. PBC diagnosis was based on the presence of antimitochondrial antibody and elevated cholestatic enzymes in all 17 patients, while one third (5/17; 29.4%) experienced severe pruritus many years before diagnosis. Patients with PBC and HBV were significantly younger at diagnosis of PBC compared to patients with PBC and HCV (56.1 ± 11.2 vs 68.5 ± 10.3, respectively, P < 0.05). At initial clinical and histological assessment the majority of patients were cirrhotics (10/17; 58.8%) with the group of PBC and HCV carrying the highest frequency (87.5% vs 33.3% in PBC and HBV; P < 0.05). The patients with HBV and concomitant PBC seem to have better outcome compared to those with HCV and PBC since none of the 6 non-cirrhotics with HBV and PBC developed cirrhosis during follow-up.

CONCLUSION: PBC diagnosis in HBV or HCV patients is very difficult and usually delayed. Therefore, in any case, cholestasis should alert physicians to further search for PBC.

• Antimitochindrial antibodies
• Autoimmune liver disease
• Hepatitis B virus
• Hepatitis C virus
• Primary biliary cirrhosis.

Primary biliary cirrhosis (PBC) is a progressive cholestatic liver disease characterized serologically by cholestasis and the presence of high-titre antimitochondrial antibodies and histologically by chronic nonsuppurative cholangitis and granulomata. PBC patients often have concomitant autoimmune diseases, including arthropathies. This raises the question as to whether there are shared features in the pathogenesis of those diseases with the pathogenesis of PBC. Epidemiological and large case studies have indicated that although the incidence of rheumatoid arthritis (RA) is not significantly raised in PBC patients, there appears to be a higher rate of RA in PBC patients and their relatives. Genetic studies have demonstrated that several genes implicated in PBC have also been implicated in RA. Epigenetic studies provided a wealth of data regarding RA, but the findings on epigenetic changes in PBC are very limited. As well, certain infectious agents identified in the pathogenesis of PBC may also play a role in the pathogenesis of RA. These data suggest that although RA is not significantly present in PBC, some individuals with certain genetic traits and environmental exposures may develop both conditions. This concept may also apply to other concomitant diseases found in PBC patients.

• Adolescent
• Adult
• Age Factors
• Aged
• Aged, 80 and over
• Autoantibodies/blood
• Autoantibodies/immunology
• Biomarkers/blood
• Celiac Disease/blood
• Celiac Disease/complications
• Celiac Disease/diagnosis
• Celiac Disease/immunology
• Child
• Chronic Disease
• Female
• Gliadin/blood
• Gliadin/immunology
• Histocompatibility Testing
• Humans
• Immunoglobulin A/blood
• Immunoglobulin A/immunology
• Liver Diseases/blood
• Liver Diseases/complications
• Liver Diseases/diagnosis
• Liver Diseases/immunology
• Male
• Middle Aged
• Transglutaminases/blood
• Transglutaminases/immunology

Primary biliary cirrhosis (PBC) is a chronic, progressive, cholestatic, organ-specific autoimmune disease of unknown etiology. It predominantly affects middle-aged women, and is characterized by autoimmune-mediated destruction of small- and medium-size intrahepatic bile ducts, portal inflammation and progressive scarring, which without proper treatment can ultimately lead to fibrosis and hepatic failure. Serum autoantibodies are crucial tools for differential diagnosis of PBC. While it is currently accepted that antimitochondrial antibodies are the most important serological markers of PBC, during the last five decades more than sixty autoantibodies have been explored in these patients, some of which had previously been thought to be specific for other autoimmune diseases.

• Primary biliary cirrhosis
• Autoimmune disease
• Autoantibody
• Anti-mitochondrial antibody
• Anti-gp210 antibody
• Anti-sp100 antibody
• Anti-centromere antibodies

Primary biliary cirrhosis (PBC) is associated with immune-mediated dermatologic disorders. The association of PBC with erythema nodosum (EN) seems rare. We report two females (42 and 44 years old) with low-grade fever, arthralgias, and elevated cholestatic enzymes in the first and fatigue in the second. Patients were also suffering from typical EN lesions characterized by multiple erythematous, painful nodules over the anterior portions of their lower extremities. Clinical and extensive laboratory work up excluded all known EN causes. PBC diagnosis was established according to the cholestatic biochemical profile, anti-mitochondrial antibodies (AMA) positivity and liver histology (first), and AMA and antinuclear (ANA) PBC-specific antibodies (second). Our report may suggest that PBC could be kept in mind in EN patients of unknown aetiology and particularly, when middle-aged female patients are affected. In such cases a thorough evaluation for AMA and/or ANA PBC-specific antibodies could be helpful to achieve a correct and timely diagnosis.