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Giorgina Mielivergani - One of the best experts on this subject based on the ideXlab platform.
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update on autoimmune hepatitis
Journal of clinical and translational hepatology, 2015Co-Authors: Rodrigo Liberal, D Vergani, Giorgina MieliverganiAbstract:Autoimmune hepatitis (AIH), a liver disorder affecting both children and adults, is characterized by inflammatory liver histology, elevated transaminase levels, circulating nonorganspecific autoantibodies, and increased levels of immunoglobulin G, in the absence of a known etiology. Two types of AIH are recognized according to seropositivity: smooth Muscle Antibody and/or antinuclear Antibody define AIH type 1 and antibodies to liver-kidney microsome type 1 and/or liver cytosol type 1 define AIH type 2. AIH type 1 affects both adults and children, while AIH type 2 is mainly a paediatric disease, though it does occasionally affects young adults. AIH should be considered during the diagnostic workup of any patient with increased liver enzyme levels. AIH is exquisitely responsive to immunosuppressive treatment with prednisolone with or without azathioprine, with symptom free long-term survival for the majority of patients. For those who do not respond to standard treatment, or who are difficult-to-treat, mycophenolate mofetil and, in the absence of a response, calcineurin inhibitors should be tried in addition to steroids. The pathogenesis of AIH is not fully understood, although there is mounting evidence that genetic susceptibility, molecular mimicry and impaired immunoregulatory networks contribute to the initiation and perpetuation of the autoimmune attack. Liver damage is thought to be mediated primarily by CD4 T-cells, although recent studies support the involvement of diverse populations, including Th17 cells. A deeper understanding of the pathogenesis of AIH is likely to contribute to the development of novel treatments, such as the adoptive transfer of autologous expanded antigenspecific regulatory T-cells, which ultimately aim at restoring tolerance to liver-derived antigens.
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autoAntibody and human leukocyte antigen profiles in children with autoimmune liver disease and their first degree relatives
Journal of Pediatric Gastroenterology and Nutrition, 2014Co-Authors: Pengyun Wang, James A. Underhill, E T Davies, D Vergani, Maria Serena Longhi, Laura J Blackmore, Tassos Grammatikopoulos, Elizabeth V Okokon, Giorgina MieliverganiAbstract:Objective: Familial clustering of juvenile autoimmune liver disease (AILD), including autoimmune hepatitis and autoimmune sclerosing cholangitis (ASC), is rare, despite a high prevalence of autoimmune disorders in AILD families. Methods: To investigate this discrepancy, we measured autoantibodies diagnostic for AILD, anti-nuclear, anti-smooth Muscle, anti-liver kidney microsomal type 1, anti-liver cytosol type 1, and anti-soluble liver antigen antibodies, and human leukocyte antigen profiles in 31 patients and 65 of their first-degree relatives (FDR). The autoAntibody profile was compared with that of 42 healthy subjects (HS). Results: Autoantibodies were detected in 71% (22/31) patients. Antinuclear Antibody or anti-smooth Muscle Antibody were present in 4/65 FDR (6.2%). HS were negative for all autoantibodies. The frequencies of homozygous HLA DRB1� 0301 (DR3) genes and haplotype A1-B8-DR3 were higher in the patients (25% and 43%) than in FDR (9% and 27%) and HS (0% and 16%). The frequencies of disease-protective genes DR4 and/or DR15 were lower in the patients (25%) than in FDR (42%) and HS (42%). Only 1 family contained 2 patients with AILD, 1 with ASC and 1 with primary sclerosing cholangitis. Both patients possessed A1-B8-DR3 genes, the ASC being homozygous and the primary sclerosing cholangitis heterozygous. Six FDR had nonhepatic autoimmune disorders, none being autoAntibody positive. Conclusions: Homozygosity for DR3 plays a major role in the predisposition to juvenile AILD. Diagnostic autoantibodies for AILD are rare among patients’ FDR and not linked to clinical manifestation of AILD.
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autoantibodies and their antigens in autoimmune hepatitis
Seminars in Liver Disease, 2009Co-Authors: Dimitrios P Bogdanos, Giorgina Mielivergani, D VerganiAbstract:AutoAntibody detection assists in the diagnosis and allows differentiation of autoimmune hepatitis (AIH) type 1 (AIH-1), characterized by antinuclear Antibody (ANA) and/or smooth Muscle Antibody (SMA), and type 2 (AIH-2), distinguished by the presence of antibodies to liver-kidney microsome type 1 (anti-LKM1) and/or antibodies to liver cytosol type 1 (anti-LC1). Detection of atypical perinuclear antineutrophil cytoplasmic antibodies (pANCA) and anti-soluble liver antigen (SLA) antibodies can act as an additional pointer toward the diagnosis of AIH, particularly in the absence of the conventional autoantibodies. Routine autoAntibody testing by indirect immunofluorescence has been recently complemented by molecular assays based on purified or recombinant antigens. Although the AIH-1-specific ANA and SMA targets need better definition, those of anti-LKM1 and anti-LC1 in AIH-2 have been clearly identified; the fine specificity of Antibody reactivity and its clinical relevance to disease pathogenesis are the focus of ongoing investigation. This article critically discusses the current knowledge of the diagnostic and clinical significance of AIH-related autoAntibody reactivities, focusing on key issues that the physician needs to be aware of to be able to request the appropriate testing and to interpret correctly the laboratory results within the clinical context of the patient.
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autoimmune hepatitis in children what is different from adult aih
Seminars in Liver Disease, 2009Co-Authors: Giorgina Mielivergani, D VerganiAbstract:Autoimmune hepatitis (AIH) is characterized by inflammatory liver histology, circulating non-organ-specific autoantibodies, and increased levels of immunoglobulin (Ig) G in the absence of a known etiology. Two types of childhood AIH are recognized according to seropositivity: smooth Muscle Antibody (SMA) and/or antinuclear Antibody (ANA), which is AIH type 1; and antibodies to liver-kidney microsome type 1 (anti-LKM1), which is AIH type 2. There is a female predominance in both. Autoimmune hepatitis type 2 presents more acutely, at a younger age, and commonly with IgA deficiency; however, duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the two groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to nonadherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AIH type 1 patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. The differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis, liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress.
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organ and non organ specific autoAntibody titres and igg levels as markers of disease activity a longitudinal study in childhood autoimmune liver disease
Autoimmunity, 2002Co-Authors: Germana V. Gregorio, D Vergani, B M Mcfarlane, Paul Bracken, Giorgina MieliverganiAbstract:No longitudinal study has investigated whether autoAntibody titres and serum IgG levels correlate with disease activity in autoimmune liver disease. To determine this, we investigated prospectively 19 patients on 254 occasions between 10 months to 5 years from diagnosis. Nine had anti-nuclear and/or anti-smooth Muscle Antibody (ANA/SMA) positive autoimmune hepatitis (type 1 AIH), 5 liver kidney microsomal type 1 (LKM-1) positive AIH (type 2 AIH) and 5 ANA/SMA positive autoimmune sclerosing cholangitis (ASC). Correlation between IgG levels, titres of ANA, SMA and LKM-1 and levels of the organ specific autoantibodies anti-liver specific protein (anti-LSP), and anti-asialoglycoprotein receptor (anti-ASGPR) with biochemical evidence of disease activity, as measured by serum aspartate amino transferase (AST) levels, was sought during the course of the disease. AST levels correlated with levels of anti-LSP, anti-ASGPR and IgG in type 1 and 2 AIH, but not in ASC. Positive correlation with AST was also observed f...
Wendy Pollock - One of the best experts on this subject based on the ideXlab platform.
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‘Actin-reactive’ discriminated from ‘non-actin-reactive’ smooth Muscle autoAntibody by immunofluorescence reactivity with rat epithelial cell line
Pathology, 2010Co-Authors: Roberta Taylor, Sharon Dearden, Cassandra C. Gill, Carol Buchner, Wendy Pollock, Rufus W. BurlingameAbstract:Aims: To compare smooth Muscle Antibody (SMA) patterns in tissue sections with patterns in an immunofluorescence assay (IFA) using a rat intestinal epithelial cell line and results from an F-actin IgG ELISA.Methods: SMA positive sera (n = 188) were classified by immunofluorescence staining of rodent kidney, stomach and liver sections as SMA-T (tubules) (n = 124) or SMA-V (vessels) (n = 64). The F-actin pattern on the rat epithelial cell line was identified by immunofluorescence staining of actin cables that was confirmed by dual immunofluorescence co-localisation with phalloidin.Results: Of 124 SMA-T positive sera, 123 reacted with the epithelial cell line and 120 with F-actin by ELISA, giving sensitivity for detection of anti-F-actin Antibody of 99% and 97%, respectively. Of 64 SMA-V positive sera, four reacted with the epithelial cell line (6%) and 41 with F-actin by ELISA (64%). Tests of 493 normal blood donors and 100 disease controls yielded specificities of 584/593 (98.5%) and 562/593 (94.8%) for th...
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A novel immunofluorescence test for f-actin-reactive smooth Muscle Antibody
Pathology, 2010Co-Authors: Ban-hock Toh, Roberta Taylor, Sharon Dearden, Cassandra C. Gill, Carol Buchner, Wendy Pollock, Rufus W. BurlingameAbstract:Aim To compare smooth Muscle Antibody (SMA) immunofluorescence staining patterns with an immunofluore-scence assay (IFA) using a rat intestinal epithelial cell line and with an F-actin ELISA. Methods SMA sera (n = 188) were classified as SMA-T (n = 124) or SMA-V (n = 64) by immunofluorescence reactivity on composite rodent tissue sections of stomach, liver and kidney. Immunofluorsecence reactivity with the rat intestinal epithelial cell line was identified by staining of linear ‘actin cables’ that was confirmed by dual immunof-luorescence co-localisation with F-actin specifically labelled by phalloidin. Results Of 124 SMA-T positive sera, 123 reacted with the rat intestinal epithelial cell line and 120 with F-actin by ELISA, giving sensitivities for detection of anti-F-actin Antibody of 99% and 97%, respectively. In contrast, only four of 64 SMA-V positive sera reacted with the rat intestinal epithelial cell line (6%) while 41 reacted with F-actin by ELISA (64%). Tests of 493 normal blood donors and 100 disease controls gave specificities of 584/593 (98.5%) and 561/593 (94.6%) for the cell line IFA and F-actin ELISA respectively. Conclusions The rat intestinal epithelial cell line im-munofluorescence is a robust diagnostic assay for anti-F-actin Antibody that can either replace the routine screening for anti-actin SMA-T Antibody in tissue sections or be used as a confirmatory assay for anti-F-actin Antibody after screening by F-actin ELISA or by immunofluorescence on rodent tissue sections.
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F-actin reactive smooth Muscle Antibody in subjects with normal liver function
Pathology, 2009Co-Authors: Ban-hock Toh, Roberta Taylor, Wendy PollockAbstract:Summary Aims Smooth Muscle Antibody (SMA) with F-actin reactivity has been reported as a diagnostic marker of autoimmune hepatitis. We re-visited this relationship by randomly selecting SMA-positive sera to test for reactivity with F-actin by ELISA. We correlated such reactivity with liver function tests Methods Sera positive for SMA by indirect immunofluorescence were tested for reactivity by F-actin ELISA and the results correlated with liver function tests. Results 89 SMA-positive sera reacted with F-actin by ELISA. Of these, 35 (39%) had normal liver enzymes, while 54 (60%) had elevated liver enzymes. There was no difference between the groups with respect to age at presentation, female preponderance or presence of anti-nuclear Antibody. In both groups, high titre SMA Antibody predominantly with immunofluorescence staining of renal glomeruli and peritubular fibrils of renal tubules (‘G/T’ subset) correlated with makedly elevated F-actin values by ELISA. ‘Actin cables’ by immunofluorescence staining of Hep-2 cells were infrequently found in both groups. Conclusion This is the first report of SMA with F-actin reactivity in subjects with normal liver function
Ban-hock Toh - One of the best experts on this subject based on the ideXlab platform.
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A novel immunofluorescence test for f-actin-reactive smooth Muscle Antibody
Pathology, 2010Co-Authors: Ban-hock Toh, Roberta Taylor, Sharon Dearden, Cassandra C. Gill, Carol Buchner, Wendy Pollock, Rufus W. BurlingameAbstract:Aim To compare smooth Muscle Antibody (SMA) immunofluorescence staining patterns with an immunofluore-scence assay (IFA) using a rat intestinal epithelial cell line and with an F-actin ELISA. Methods SMA sera (n = 188) were classified as SMA-T (n = 124) or SMA-V (n = 64) by immunofluorescence reactivity on composite rodent tissue sections of stomach, liver and kidney. Immunofluorsecence reactivity with the rat intestinal epithelial cell line was identified by staining of linear ‘actin cables’ that was confirmed by dual immunof-luorescence co-localisation with F-actin specifically labelled by phalloidin. Results Of 124 SMA-T positive sera, 123 reacted with the rat intestinal epithelial cell line and 120 with F-actin by ELISA, giving sensitivities for detection of anti-F-actin Antibody of 99% and 97%, respectively. In contrast, only four of 64 SMA-V positive sera reacted with the rat intestinal epithelial cell line (6%) while 41 reacted with F-actin by ELISA (64%). Tests of 493 normal blood donors and 100 disease controls gave specificities of 584/593 (98.5%) and 561/593 (94.6%) for the cell line IFA and F-actin ELISA respectively. Conclusions The rat intestinal epithelial cell line im-munofluorescence is a robust diagnostic assay for anti-F-actin Antibody that can either replace the routine screening for anti-actin SMA-T Antibody in tissue sections or be used as a confirmatory assay for anti-F-actin Antibody after screening by F-actin ELISA or by immunofluorescence on rodent tissue sections.
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F-actin reactive smooth Muscle Antibody in subjects with normal liver function
Pathology, 2009Co-Authors: Ban-hock Toh, Roberta Taylor, Wendy PollockAbstract:Summary Aims Smooth Muscle Antibody (SMA) with F-actin reactivity has been reported as a diagnostic marker of autoimmune hepatitis. We re-visited this relationship by randomly selecting SMA-positive sera to test for reactivity with F-actin by ELISA. We correlated such reactivity with liver function tests Methods Sera positive for SMA by indirect immunofluorescence were tested for reactivity by F-actin ELISA and the results correlated with liver function tests. Results 89 SMA-positive sera reacted with F-actin by ELISA. Of these, 35 (39%) had normal liver enzymes, while 54 (60%) had elevated liver enzymes. There was no difference between the groups with respect to age at presentation, female preponderance or presence of anti-nuclear Antibody. In both groups, high titre SMA Antibody predominantly with immunofluorescence staining of renal glomeruli and peritubular fibrils of renal tubules (‘G/T’ subset) correlated with makedly elevated F-actin values by ELISA. ‘Actin cables’ by immunofluorescence staining of Hep-2 cells were infrequently found in both groups. Conclusion This is the first report of SMA with F-actin reactivity in subjects with normal liver function
Rufus W. Burlingame - One of the best experts on this subject based on the ideXlab platform.
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‘Actin-reactive’ discriminated from ‘non-actin-reactive’ smooth Muscle autoAntibody by immunofluorescence reactivity with rat epithelial cell line
Pathology, 2010Co-Authors: Roberta Taylor, Sharon Dearden, Cassandra C. Gill, Carol Buchner, Wendy Pollock, Rufus W. BurlingameAbstract:Aims: To compare smooth Muscle Antibody (SMA) patterns in tissue sections with patterns in an immunofluorescence assay (IFA) using a rat intestinal epithelial cell line and results from an F-actin IgG ELISA.Methods: SMA positive sera (n = 188) were classified by immunofluorescence staining of rodent kidney, stomach and liver sections as SMA-T (tubules) (n = 124) or SMA-V (vessels) (n = 64). The F-actin pattern on the rat epithelial cell line was identified by immunofluorescence staining of actin cables that was confirmed by dual immunofluorescence co-localisation with phalloidin.Results: Of 124 SMA-T positive sera, 123 reacted with the epithelial cell line and 120 with F-actin by ELISA, giving sensitivity for detection of anti-F-actin Antibody of 99% and 97%, respectively. Of 64 SMA-V positive sera, four reacted with the epithelial cell line (6%) and 41 with F-actin by ELISA (64%). Tests of 493 normal blood donors and 100 disease controls yielded specificities of 584/593 (98.5%) and 562/593 (94.8%) for th...
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A novel immunofluorescence test for f-actin-reactive smooth Muscle Antibody
Pathology, 2010Co-Authors: Ban-hock Toh, Roberta Taylor, Sharon Dearden, Cassandra C. Gill, Carol Buchner, Wendy Pollock, Rufus W. BurlingameAbstract:Aim To compare smooth Muscle Antibody (SMA) immunofluorescence staining patterns with an immunofluore-scence assay (IFA) using a rat intestinal epithelial cell line and with an F-actin ELISA. Methods SMA sera (n = 188) were classified as SMA-T (n = 124) or SMA-V (n = 64) by immunofluorescence reactivity on composite rodent tissue sections of stomach, liver and kidney. Immunofluorsecence reactivity with the rat intestinal epithelial cell line was identified by staining of linear ‘actin cables’ that was confirmed by dual immunof-luorescence co-localisation with F-actin specifically labelled by phalloidin. Results Of 124 SMA-T positive sera, 123 reacted with the rat intestinal epithelial cell line and 120 with F-actin by ELISA, giving sensitivities for detection of anti-F-actin Antibody of 99% and 97%, respectively. In contrast, only four of 64 SMA-V positive sera reacted with the rat intestinal epithelial cell line (6%) while 41 reacted with F-actin by ELISA (64%). Tests of 493 normal blood donors and 100 disease controls gave specificities of 584/593 (98.5%) and 561/593 (94.6%) for the cell line IFA and F-actin ELISA respectively. Conclusions The rat intestinal epithelial cell line im-munofluorescence is a robust diagnostic assay for anti-F-actin Antibody that can either replace the routine screening for anti-actin SMA-T Antibody in tissue sections or be used as a confirmatory assay for anti-F-actin Antibody after screening by F-actin ELISA or by immunofluorescence on rodent tissue sections.
D Vergani - One of the best experts on this subject based on the ideXlab platform.
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update on autoimmune hepatitis
Journal of clinical and translational hepatology, 2015Co-Authors: Rodrigo Liberal, D Vergani, Giorgina MieliverganiAbstract:Autoimmune hepatitis (AIH), a liver disorder affecting both children and adults, is characterized by inflammatory liver histology, elevated transaminase levels, circulating nonorganspecific autoantibodies, and increased levels of immunoglobulin G, in the absence of a known etiology. Two types of AIH are recognized according to seropositivity: smooth Muscle Antibody and/or antinuclear Antibody define AIH type 1 and antibodies to liver-kidney microsome type 1 and/or liver cytosol type 1 define AIH type 2. AIH type 1 affects both adults and children, while AIH type 2 is mainly a paediatric disease, though it does occasionally affects young adults. AIH should be considered during the diagnostic workup of any patient with increased liver enzyme levels. AIH is exquisitely responsive to immunosuppressive treatment with prednisolone with or without azathioprine, with symptom free long-term survival for the majority of patients. For those who do not respond to standard treatment, or who are difficult-to-treat, mycophenolate mofetil and, in the absence of a response, calcineurin inhibitors should be tried in addition to steroids. The pathogenesis of AIH is not fully understood, although there is mounting evidence that genetic susceptibility, molecular mimicry and impaired immunoregulatory networks contribute to the initiation and perpetuation of the autoimmune attack. Liver damage is thought to be mediated primarily by CD4 T-cells, although recent studies support the involvement of diverse populations, including Th17 cells. A deeper understanding of the pathogenesis of AIH is likely to contribute to the development of novel treatments, such as the adoptive transfer of autologous expanded antigenspecific regulatory T-cells, which ultimately aim at restoring tolerance to liver-derived antigens.
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autoAntibody and human leukocyte antigen profiles in children with autoimmune liver disease and their first degree relatives
Journal of Pediatric Gastroenterology and Nutrition, 2014Co-Authors: Pengyun Wang, James A. Underhill, E T Davies, D Vergani, Maria Serena Longhi, Laura J Blackmore, Tassos Grammatikopoulos, Elizabeth V Okokon, Giorgina MieliverganiAbstract:Objective: Familial clustering of juvenile autoimmune liver disease (AILD), including autoimmune hepatitis and autoimmune sclerosing cholangitis (ASC), is rare, despite a high prevalence of autoimmune disorders in AILD families. Methods: To investigate this discrepancy, we measured autoantibodies diagnostic for AILD, anti-nuclear, anti-smooth Muscle, anti-liver kidney microsomal type 1, anti-liver cytosol type 1, and anti-soluble liver antigen antibodies, and human leukocyte antigen profiles in 31 patients and 65 of their first-degree relatives (FDR). The autoAntibody profile was compared with that of 42 healthy subjects (HS). Results: Autoantibodies were detected in 71% (22/31) patients. Antinuclear Antibody or anti-smooth Muscle Antibody were present in 4/65 FDR (6.2%). HS were negative for all autoantibodies. The frequencies of homozygous HLA DRB1� 0301 (DR3) genes and haplotype A1-B8-DR3 were higher in the patients (25% and 43%) than in FDR (9% and 27%) and HS (0% and 16%). The frequencies of disease-protective genes DR4 and/or DR15 were lower in the patients (25%) than in FDR (42%) and HS (42%). Only 1 family contained 2 patients with AILD, 1 with ASC and 1 with primary sclerosing cholangitis. Both patients possessed A1-B8-DR3 genes, the ASC being homozygous and the primary sclerosing cholangitis heterozygous. Six FDR had nonhepatic autoimmune disorders, none being autoAntibody positive. Conclusions: Homozygosity for DR3 plays a major role in the predisposition to juvenile AILD. Diagnostic autoantibodies for AILD are rare among patients’ FDR and not linked to clinical manifestation of AILD.
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autoantibodies and their antigens in autoimmune hepatitis
Seminars in Liver Disease, 2009Co-Authors: Dimitrios P Bogdanos, Giorgina Mielivergani, D VerganiAbstract:AutoAntibody detection assists in the diagnosis and allows differentiation of autoimmune hepatitis (AIH) type 1 (AIH-1), characterized by antinuclear Antibody (ANA) and/or smooth Muscle Antibody (SMA), and type 2 (AIH-2), distinguished by the presence of antibodies to liver-kidney microsome type 1 (anti-LKM1) and/or antibodies to liver cytosol type 1 (anti-LC1). Detection of atypical perinuclear antineutrophil cytoplasmic antibodies (pANCA) and anti-soluble liver antigen (SLA) antibodies can act as an additional pointer toward the diagnosis of AIH, particularly in the absence of the conventional autoantibodies. Routine autoAntibody testing by indirect immunofluorescence has been recently complemented by molecular assays based on purified or recombinant antigens. Although the AIH-1-specific ANA and SMA targets need better definition, those of anti-LKM1 and anti-LC1 in AIH-2 have been clearly identified; the fine specificity of Antibody reactivity and its clinical relevance to disease pathogenesis are the focus of ongoing investigation. This article critically discusses the current knowledge of the diagnostic and clinical significance of AIH-related autoAntibody reactivities, focusing on key issues that the physician needs to be aware of to be able to request the appropriate testing and to interpret correctly the laboratory results within the clinical context of the patient.
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autoimmune hepatitis in children what is different from adult aih
Seminars in Liver Disease, 2009Co-Authors: Giorgina Mielivergani, D VerganiAbstract:Autoimmune hepatitis (AIH) is characterized by inflammatory liver histology, circulating non-organ-specific autoantibodies, and increased levels of immunoglobulin (Ig) G in the absence of a known etiology. Two types of childhood AIH are recognized according to seropositivity: smooth Muscle Antibody (SMA) and/or antinuclear Antibody (ANA), which is AIH type 1; and antibodies to liver-kidney microsome type 1 (anti-LKM1), which is AIH type 2. There is a female predominance in both. Autoimmune hepatitis type 2 presents more acutely, at a younger age, and commonly with IgA deficiency; however, duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the two groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to nonadherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AIH type 1 patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. The differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis, liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress.
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organ and non organ specific autoAntibody titres and igg levels as markers of disease activity a longitudinal study in childhood autoimmune liver disease
Autoimmunity, 2002Co-Authors: Germana V. Gregorio, D Vergani, B M Mcfarlane, Paul Bracken, Giorgina MieliverganiAbstract:No longitudinal study has investigated whether autoAntibody titres and serum IgG levels correlate with disease activity in autoimmune liver disease. To determine this, we investigated prospectively 19 patients on 254 occasions between 10 months to 5 years from diagnosis. Nine had anti-nuclear and/or anti-smooth Muscle Antibody (ANA/SMA) positive autoimmune hepatitis (type 1 AIH), 5 liver kidney microsomal type 1 (LKM-1) positive AIH (type 2 AIH) and 5 ANA/SMA positive autoimmune sclerosing cholangitis (ASC). Correlation between IgG levels, titres of ANA, SMA and LKM-1 and levels of the organ specific autoantibodies anti-liver specific protein (anti-LSP), and anti-asialoglycoprotein receptor (anti-ASGPR) with biochemical evidence of disease activity, as measured by serum aspartate amino transferase (AST) levels, was sought during the course of the disease. AST levels correlated with levels of anti-LSP, anti-ASGPR and IgG in type 1 and 2 AIH, but not in ASC. Positive correlation with AST was also observed f...
