ABSTRACT
Anti-mitochondrial antibody (AMA) is a typical serum marker for primary biliary cholangitis (PBC), and the diagnosis of AMA-negative PBC may easily be neglected in clinical practice. This article analyzes the differences in epidemiology, clinical and pathological features, and treatment outcome between AMA-negative and AMA-positive PBC and points out that there are significant differences between them in the symptom of pruritus, immunoglobulin M, and severity of bile duct injury. For patients with a clinical diagnosis of AMA-negative PBC, immunofluorescence assay combined with immunological detection should be performed to exclude the false-negative result of AMA, and a confirmed diagnosis can be made with reference to the manifestation of cholestasis, positive results of anti-sp100, anti-gp210, and anti-ANA antibodies, and typical hyperactive cholangitis based on pathological examination. Differential diagnosis of this disease with other types of bile duct injury or absence of bile duct should be taken seriously in clinical practice.
ABSTRACT
Anti-mitochondrial antibody (AMA) is a typical serum marker for primary biliary cholangitis (PBC), and the diagnosis of AMA-negative PBC may easily be neglected in clinical practice. This article analyzes the differences in epidemiology, clinical and pathological features, and treatment outcome between AMA-negative and AMA-positive PBC and points out that there are significant differences between them in the symptom of pruritus, immunoglobulin M, and severity of bile duct injury. For patients with a clinical diagnosis of AMA-negative PBC, immunofluorescence assay combined with immunological detection should be performed to exclude the false-negative result of AMA, and a confirmed diagnosis can be made with reference to the manifestation of cholestasis, positive results of anti-sp100, anti-gp210, and anti-ANA antibodies, and typical hyperactive cholangitis based on pathological examination. Differential diagnosis of this disease with other types of bile duct injury or absence of bile duct should be taken seriously in clinical practice.
ABSTRACT
La hepatitis autoinmune es una inflamación hepatocelular que se caracteriza por diversos autoanticuerpos circulantes. En el presente trabajo se evaluó la concordancia entre los resultados obtenidos por la técnica ELISA y por la técnica de inmunofluorescencia indirecta (IFI) para la determinación de autoanticuerpos en hepatitis autoinmune. Se incluyeron 123 pacientes con hepatitis autoinmune, 91 (74%) del sexo femenino y 32 (26%) de sexomasculino, mayores de 18 años, en los cuales se realizó un estudio comparativo entre ELISA e inmunofluorescencia indirecta para la detección de los anticuerpos antinucleares (78 pacientes), anticuerpos antimitocondriales (84pacientes) y antimicrosoma hepatorrenal (85 pacientes). De acuerdo al valor kappa obtenido se encontró que para el anticuerpo antimicrosoma hepatorrenalel nivel de concordancia fue muy bueno (k=1,0, p<0,001); para el anticuerpoantinuclear el nivel de concordancia fue débil, sin embargo fue significativo(k=0,37, p<0,001) mientras que para el anticuerpo antimitocondrialel nivel de concordancia fue pobre (k=0,05, p<0,476). La determinación del anticuerpo antimicrosoma hepatorrenal fue la prueba con mayor sensibilidad, especificidad y concordancia entre ambas técnicas analizadas y se estableció que la técnica de ELISA para el anticuerpo antimitocondrial solo concuerda con la técnica de inmunofluorescencia indirecta en sujetos con títulos altos.
Autoimmune hepatitis is a hepatocellular inflammation which is characterized by different circulating autoantibodies. In this paper, the correlation between the results obtained by ELISA and indirect immunofluorescence technique for the determination of autoantibodies in autoimmune hepatitis was evaluated. One hundred and twenty-three patients with autoimmune hepatitis, 91 (74%) female and 32 (26%) male, over 18 years were included. In these patients, a comparative study between ELISA and indirect immunofluorescence detection of the antinuclear antibodies (78 patients), anti-mitochondrial antibodies (84 patients) and anti-liver kidney microsome (85 patients) was performed. According to the kappa value obtained, it was found that for anti-liver kidney microsome the level of agreement was very good (k=1.0, p<0.001) for antinuclear antibody, the level of concordance was weak but significant (k=0.37, p<0.001,) meanwhile antimitochondrial antibody level of concordance was poor (k=0.05, p<0.476).The determination of the antibody anti-liver kidney microsome was the test with greater sensitivity, specificity and concordance between the two techniques discussed and it was established that the ELISA technique for antimitochondrial antibody is only in concordance with the indirect immunofluorescence in patients with high titers.
A hepatite autoimune é uma inflamação hepatocelular, que se caracteriza pelos diferentes anticorpos circulantes. Neste trabalho foi avaliada a correlação entre os resultados obtidos através da técnica ELISA e da técnica de imunofluorescência indireta (IFI) para a determinação de auto-anticorpos na hepatite auto-imune. Foram incluídos 123 pacientes com hepatite autoimune, 91 (74%) do sexo feminino e 32 (26%) do sexo masculino, maiores de 18 anos, nos quais foi realizado um estudo comparativo entre ELISA e Imunofluoresência Indireta para a detecção dos anticorpos antinucleares (78 pacientes), anticorpos antimitocondriais (84 pacientes) e antimicrossomal hepatorrenal (85 pacientes). De acordo ao valor kappa obtido, foi encontrado que para o anticorpo antimicrossomal hepatorrenal o nivel de concordância foi muito bom (k=1,0, p<0,001); para o anticorpo antinuclear o nivel de concordância foi fraco, porém foi significativo (k=0,37, p<0,001) enquanto que para o anticorpo antimitocondrial o nivel de concordância foi pobre (k=0,05, p<0,476). A determinação do anticorpo antimicrossomal hepatorrenal foi o teste com maior sensibilidade, especificidade e concordância entre ambas técnicas analisadas e se estabeleceu que a técnica de ELISA, para o anticorpo antimitocondrial, só concorda com a técnica de Imunofluorescência Indireta em sujeitos com títulos altos.
Subject(s)
Humans , Male , Female , Autoantibodies , Hepatitis, Autoimmune/etiology , Antibodies, Antinuclear , Enzyme-Linked Immunosorbent Assay , Fluorescent Antibody Technique, Indirect , Hepatitis , LiverABSTRACT
BACKGROUND: Cytoplasmic stainings in antinuclear antibody tests using HEp-2 cells are usually undetermined and the significance has not been fully understood until now. Hence, we evaluated their clinical characteristics and also the coexistence of other autoantibodies in the sera with cytoplasmic stainings in antinuclear antibody tests. METHODS: We reviewed clinical records retrospectively in 53 sera showing cytoplasmic stainings among 3, 610 sera that were tested antinuclear antibodies from January to September, 2002 and performed antimitochondrial antibodies (AMA) tests using indirect immunofluorescence (IIF) and antibodies to antiribosomal P and extractable nuclear antigens (ENA) tests using enzyme immunoassay (EIA). RESULTS: Among 53 sera with cytoplasmic stainings, 31 sera showed an AMA pattern and 15 sera showed an antibody to ribosomal P pattern. Three cytoskeletal and one golgi complex patterns were also observed. The most common diagnosis was autoimmune disorders (32, 60.4%) and hepatic disorders (excluding autoimmune hepatitis) (6, 11.3%). Hepatic disorders including autoimmune, drug-induced, and alcoholic hepatitis were most commonly observed (32.3%) in sera with an AMA pattern. On the other hand, various autoimmune disorders such as SLE, systemic sclerosis, dermatomyositis, and polymyositis were observed (86.7%) in sera with a ribosomal P pattern. Of 31 sera with the AMA pattern, the corresponding antibodies were confirmed in three by IIF and of 15 sera with a ribosomal P pattern, only one was confirmed to have this antibody by EIA. All the confirmed sera showed high titered (>1: 320) cytoplasmic stainings. Antibodies to ENA were positive in sixteen (RnP, 5; Sm, 4; Ro, 5; La, 2) and anti-DNA in three of the sera. CONCLUSIONS: Although cytoplasmic staining patterns are not disease specific, it is suggested that continuous high titer stainings be followed up since they could provide diagnostic help.
Subject(s)
Antibodies , Antibodies, Antinuclear , Antigens, Nuclear , Autoantibodies , Cytoplasm , Dermatomyositis , Diagnosis , Fluorescent Antibody Technique, Indirect , Golgi Apparatus , Hand , Hepatitis, Alcoholic , Immunoenzyme Techniques , Polymyositis , Retrospective Studies , Scleroderma, SystemicABSTRACT
Primary biliary cirrhosis (PBC) is a chronic progressive disease, more often affecting women, characterised by progressive destruction of the small intrahepatic bile ducts with portal inflammation leading to fibrosis and cirrhosis. There is a close association between PBC and antibodies to antimitochondrial antibodies (AMA). The pathogenesis of PBC remains uncertain. The disease has been considered as an example of autoimmunity. Estimates of disease prevalence vary between 20 and 240 cases per milion per year. PBC is a extremely rare liver disease in Asia. There is no definitive drug treatment, liver transplantation is indicated for patients with intractable symptoms and for end-stage disease. The authors have experienced a case of PBC in 66 year-old female who suffered from lethargy, pruritus and dry mouth since 2 years ago. We report a case of PBC with a review of literatures.