Update of the Brazilian Society of Hepatology Recommendations for Diagnosis and Management of Autoimmune Diseases of the Liver

Couto, Cláudia Alves; Cançado, Eduardo Luiz Rachid; Porta, Gilda; Levy, Cynthia; Silva, Antônio Eduardo Benedito; Bittencourt, Paulo Lisboa; Carvalho-Filho, Roberto José de; Chaves, Dalton Marques; Miura, Irene Kazue; Codes, Liana; Faria, Luciana Costa; Evangelista, Ana Catarina Jorge; Farias, Alberto Queiróz; Gonçalves, L. L.; Harriz, Michelle; Lopes, Edmundo Pessoa; Luz, Gustavo O.; Oliveira, Patrícia; Oliveira, Elze Maria Gomes; Narciso-Schiavon, Janaína Luz; Sevá‐Pereira, Tiago

doi:10.1590/s0004-2803.201900000-43

Cited by 8 publications

(6 citation statements)

References 45 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…14 The diagnosis is usually a challenge, and the Paris Criteria are the most widely chosen tool. 7,[14][15][16] Many patients are initially diagnosed with PBC and treated with UDCA, but subsequent raises in aminotransferases and gamma-globulin, as well as a poor response to therapy, lead the physicians to suspect an AIH-PBC-OS and change the treatment or perform a liver biopsy. 14 Although our patient presented with a cholestatic pattern of liver injury, our team decided to start prednisone considering the autoantibodies profile and numerous foci of lobular necrosis, with no relevant biliary changes in the first histologic evaluation of the liver.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

et al. 2023

View full text Add to dashboard Cite

COVID-19 is commonly associated with high serum levels of pro-inflammatory cytokines, and the post-infection status can disturb self-tolerance and trigger autoimmune responses. We are reporting a 45-year-old male who was admitted with fatigue, jaundice, elevated liver enzymes (with cholestatic pattern), and acute kidney injury two weeks after recovering from a mild SARS-CoV-2 infection. Serologies for viral hepatitis and anti-mitochondrial antibody were negative, while anti-nuclear and anti-smooth muscle antibodies were positive. There were no signs of chronic liver disease, and a magnetic resonance cholangiography showed no dilatation of biliary ducts. Histologic evaluation of the liver evidenced numerous foci of lobular necrosis without ductopenia or portal biliary reaction. Considering the autoantibody profile and histologic changes, the medical team started oral prednisone, but there was a suboptimal biochemical response in the outpatient follow-up. Two months later, a second liver biopsy was performed and revealed non-suppurative destructive chronic cholangitis, extensive areas of confluent necrosis with hepatocytes regenerating into pseudorosettes, and numerous plasma cells. According to the Paris Criteria, the patient was then diagnosed with an autoimmune hepatitis-primary biliary cholangitis overlap syndrome (AIH-PBC-OS). After adding azathioprine and ursodeoxycholic acid to the treatment, there was a satisfactory response. This is the second worldwide report of an AIH-PBC-OS triggered by COVID-19, but the first case with a negative anti-mitochondrial antibody. In this setting, histologic evaluation of the liver by an experienced pathologist is a hallmark of achieving the diagnosis and correctly treat the patient.

show abstract

Section: Discussionmentioning

confidence: 99%

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

et al. 2023

View full text Add to dashboard Cite

show abstract

“…We enrolled 55 patients with PBC who fulfilled the consensus criteria of PBC and were admitted to the Peking Union Medical College Hospital (PUMCH) from July 2017 to September 2019 (Table 1) (17). Substantial higher alkaline phosphatase (ALP), gamma-glutamyl transferase, and total bile acid were found in patients with PBC.…”

Section: Patients and Healthy Controlsmentioning

confidence: 99%

CXCL12-CXCR4-Mediated Chemotaxis Supports Accumulation of Mucosal-Associated Invariant T Cells Into the Liver of Patients With PBC

Chen

Liu

et al. 2021

Front. Immunol.

View full text Add to dashboard Cite

Objectives: To explore the potential role of CD3+CD8+CD161high TCRVα7.2+ mucosal-associated invariant T (MAIT) cells in the pathogenesis of primary biliary cholangitis (PBC).Methods: We enrolled 55 patients with PBC, 69 healthy controls (HCs), and 8 patients with hepatic hemangioma. Circulating MAIT cells and their chemokine receptor profiles and cytokine production were quantified using flow cytometry. Liver-resident MAIT cells were examined by immunofluorescence staining. CXCL12-mediated chemotaxis of MAIT cells was measured using a transwell migration assay. Plasma interleukin (IL)-18 was measured using ELISA, and cytokine production in IL-18-stimulated MAIT cells was detected using flow cytometry.Result: Peripheral MAIT cells were found to be significantly lower in patients with PBC (3.0 ± 3.2% vs. 9.4 ± 8.0%, p < 0.01) and negatively correlated with alkaline phosphatase (ALP) levels (r = −0.3209, p < 0.05). Liver immunofluorescence staining suggested that MAIT cells might accumulate in PBC liver. MAIT cells from patients with PBC expressed higher levels of CXCR4 (84.8 ± 18.0% vs. 58.7 ± 11.4%, p < 0.01), and the expression of CXCL12 was higher in PBC liver. CXCL12 promoted MAIT cell chemotaxis (70.4 ± 6.8% vs. 52.2 ± 3.5%, p < 0.01), which was attenuated by CXCR4 antagonist. MAIT cells from PBC produced significantly more interferon-γ (IFN-γ) (88.3 ± 4.2% vs. 64.2 ± 10.1%, p < 0.01), tumor necrosis factor-α (TNF-α) (93.0 ± 1.1% vs. 80.1 ± 5.3%, p < 0.01), Granzyme B (89.3 ± 3.3% vs. 72.1 ± 7.0%, p < 0.01), and perforin (46.8 ± 6.6% vs. 34.8 ± 7.7%, p < 0.05). MAIT cells from PBC expressed higher levels of IL18-Rα (83.8 ± 10.2% vs. 58.3 ± 8.7%, p < 0.01). Plasma IL-18 was more abundant in patients with PBC (286.8 ± 75.7 pg/ml vs. 132.9 ± 78.1 pg/ml, p < 0.01). IL-18 promoted IFN-γ production in MAIT cells (74.9 ± 6.6% vs. 54.7 ± 6.7%, p < 0.01), which was partially attenuated by blocking IL-18R (68.6 ± 8.3% vs. 43.5 ± 4.2%, p < 0.01).Conclusion: Mucosal-associated invariant T cells from patients with PBC accumulated in the liver via CXCL12-CXCR4-mediated chemotaxis, produced pro-inflammatory cytokines, and contributed to portal inflammation, which was potentially mediated by elevated IL-18. Targeting MAIT cells might be a therapeutic approach for PBC.

show abstract

“…Patients diagnosed with PSC between 1991 and 2021 in 23 different hepatology centers across the country were enrolled in this retrospective study. e inclusion criteria were the diagnosis of PSC, which was considered in the presence of cholestasis and compatible imaging characteristics of PSC disclosed either by magnetic resonance imaging or endoscopic retrograde cholangiography, as recommended by national and international guidelines [26][27][28]. Small duct PSC was considered in the presence of typical histological features of PSCs in patients with IBD with normal bile ducts on cholangiography [26,27].…”

Section: Study Population and Case Definitionmentioning

confidence: 99%

“…e inclusion criteria were the diagnosis of PSC, which was considered in the presence of cholestasis and compatible imaging characteristics of PSC disclosed either by magnetic resonance imaging or endoscopic retrograde cholangiography, as recommended by national and international guidelines [26][27][28]. Small duct PSC was considered in the presence of typical histological features of PSCs in patients with IBD with normal bile ducts on cholangiography [26,27]. In this study, the overlap syndrome of autoimmune hepatitis (AIH) and PSC was defined based on typical findings of PSC in patients with additional diagnostic criteria for AIH, as suggested by the International Autoimmune Study Group [29].…”

Section: Study Population and Case Definitionmentioning

confidence: 99%

Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population

Nardelli

Bittencourt

Cançado

et al. 2021

Canadian Journal of Gastroenterology and Hepatology

Self Cite

View full text Add to dashboard Cite

Background. Primary sclerosing cholangitis (PSC) is associated with a broad phenotypic spectrum in different populations from diverse ethnic and racial backgrounds. This study aimed to describe the clinical characteristics and outcomes of PSC in a multicenter cohort of patients from Brazil. Methods. Data from the Brazilian Cholestasis Study Group were retrospectively reviewed to assess demographic information and clinical characteristics of PSC, as well as the outcomes, such as transplantation-free survival. Results. This cohort included 210 patients. After excluding 33 (15.7%) patients with PSC and overlap syndrome of autoimmune hepatitis, 177 (97 males, median age 33 (21–42) years) with clear-cut PSC were eligible for this study. Most of the patients (n = 139, 78.5%) were symptomatic, and 104 (58.7%) had advanced PSC at the time of diagnosis. Concurrent inflammatory bowel disease was observed in 78 (58.6%) of the investigated patients (n = 133), and most of them had ulcerative colitis (n = 61, 78.2%). The 1- and 5-year survival free of liver transplantation or death were 92.3 ± 2.1% and 66.9 ± 4.2%, respectively, and baseline advanced PSC, pruritus, and elevated bilirubin levels were independent risk factors for the composite adverse outcome. Females were significantly older and had lower bilirubin levels than males at baseline, but survival was not associated with sex. Approximately 12.4% (n = 22) of patients with PSC died, and 32.8% (n = 58) underwent liver transplantation at a median follow-up time of 5.3 and 3.2 years. Conclusion. Multiethnic Brazilian PSC patients exhibited a less pronounced male predominance and a lower frequency of inflammatory bowel disease than Caucasians. Adverse outcomes were more frequent, probably due to advanced disease at baseline.

show abstract

Update of the Brazilian Society of Hepatology Recommendations for Diagnosis and Management of Autoimmune Diseases of the Liver

Cited by 8 publications

References 45 publications

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

CXCL12-CXCR4-Mediated Chemotaxis Supports Accumulation of Mucosal-Associated Invariant T Cells Into the Liver of Patients With PBC

Clinical Features and Outcomes of Primary Sclerosing Cholangitis in the Highly Admixed Brazilian Population

Contact Info

Product

Resources

About