Veille scientifique | Cholangite biliaire primitive (CBP), Cholangite sclérosante primitive (CSP), Hépatite auto-immune (HAI), Syndrome LPAC

Hepatic accumulation of bile acids (BAs) contributes to cholestasis-induced liver injury and fibrosis. Our previous studies have shown that Lactobacillus murinus ( L. murinus) alleviates liver fibrosis in primary sclerosing cholangitis (PSC) by modulating bile acid metabolism. In this study, we found that L. murinus intervention alleviated hepatic taurocholic acid (TCA) accumulation, cholestasis, and liver fibrosis in PSC mice. TCA was shown to promote liver fibrosis in PSC mice by enhancing M2...

Primary biliary cholangitis (PBC) is an immune-mediated cholestatic liver disease, and osteoporosis (OP) is a prevalent comorbidity that aggravates the disease burden of PBC patients. Although the co-occurrence of the 2 diseases has been widely observed, the underlying molecular mechanisms remain unclear. Herein, we evaluated the causal link between PBC and OP using Mendelian randomization (MR) and explored shared molecular mechanisms through bioinformatics and machine learning. We performed...

Autoimmune hepatitis (AIH) is a chronic progressive inflammatory liver disease with a rising global incidence. The treatment of AIH remains challenging because first-line drugs show limited efficacy and systemic side effects. Gut microbiota plays a crucial role in the pathogenesis of AIH, leading to growing interest in developing probiotic-based therapies. In this study, we used multi-omics analysis to investigate the therapeutic effects of a quadruple probiotic mixture (Probiotic-quad)...

Autoimmune liver diseases (AILDs) are considered rare conditions, though the frequency of their diagnosis is increasing thanks to the introduction of new diagnostic tests and improvements in analytical technologies. In clinical practice, in patients with liver disease of unknown etiology, after a differential diagnosis that excludes other possible causes of hepatic damage, a potential autoimmune origin should be investigated through the evaluation of a specific autoantibody panel. In this...

Interleukin-17 (IL-17) and IL-17+ secretory cells, including T helper 17 (Th17) cells play pivotal roles in autoimmune diseases such as psoriasis. Emerging evidence implicates the IL-17 pathway in primary sclerosing cholangitis (PSC), a chronic cholestatic liver disease lacking approved pharmacological therapies, for which liver transplantation remains the only life-extending option. The IL-17 pathway and Th17 biology is increasingly implicated in PSC pathogenesis through its interactions with...

Haploinsufficiency of A20 (HA20) is a primary immune regulation disease caused by heterozygous loss-of-function variants in TNFAIP3, resulting in unchecked inflammatory signaling. HA20 is a highly heterogeneous disorder with overlapping features of autoinflammation, autoimmunity, immunodeficiency, atopy, and lymphoproliferation. Most patients develop symptoms in early childhood mimicking Behcet's disease, inflammatory bowel disease, periodic fevers with aphthous stomatitis, pharyngitis, and...

CONCLUSION: ADSCs combined with prednisone synergistically ameliorate immune-mediated liver injury through dual immunosuppression and modulation of key signaling pathways, promoting immune tolerance and tissue repair. This strategy offers a promising therapeutic approach for AIH.

Long-standing inflammatory bowel disease (IBD) increases the risk of dysplasia and colorectal cancer (CRC), particularly in patients with extensive disease or coexisting primary sclerosing cholangitis (PSC). Several non-conventional dysplasia subtypes have been identified. Hypermucinous dysplasia was first formally proposed in 2020, and it arises predominantly in patients with long-standing ulcerative colitis, frequently in extensive disease and concomitant PSC. Histologically, hypermucinous...

CONCLUSION: This study provides comprehensive genomic evidence for the genetic connections between PBC and the four autoimmune diseases (MS, PS, RA, and SS), offering valuable insights into the shared pathological mechanisms underlying their comorbidities.

CONCLUSIONS: Prevalence and incidence of rPSC post-LT varied according to continents. Younger recipient age, male sex, de novo IBD, cyclosporine use post-LT and acute rejection episodes were associated with a higher risk of recurrence.

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Cytomegalovirus (CMV) is an infrequently reported etiology of secondary pouchitis in patients who have undergone total proctocolectomy with ileal pouch-anal anastomosis. We present the case of a patient with chronic antibiotic-dependent pouchitis, ulcerative pancolitis, primary sclerosing cholangitis, and orthotopic liver transplant who presented with hematochezia. Pouchoscopy showed ulcerated mucosa, and histopathology with immunohistochemistry confirmed CMV pouchitis. The patient was treated...

CONCLUSION: This study highlights the critical role of HLA-DRB1* alleles, particularly DRB1*03:01 and DRB1*04:01, in the genetic susceptibility to AIH in the Yemeni population. These findings not only enhance the understanding of genetic factors in AIH but also establish a foundation for future immunogenetic research in the Middle East. The identification of region-specific HLA associations could inform both diagnostic and therapeutic strategies for AIH in Yemen and similar populations. This...

CONCLUSIONS: MCC950 exerts a hepatoprotective effect in the Con A-induced EAH mouse model by suppressing the NLRP3 inflammasome pathway and reducing pyroptosis.

CONCLUSIONS: The influence of Lp(a) on the determination of LDL-C may lead to clinically significant overestimations of the actual LDL-C. Therefore, we recommend using Lp(a)-corrected LDL-C when 1) the Lp(a) concentration is high, 2) the LDL-C concentration is low, and 3) the LDL-C-lowering treatment is less effective than expected.

Eight new spiropolycyclic polyprenylated acylphloroglucinols, named spiropatuols A-H (1-8), were isolated from the traditional Chinese medicinal herb Hypericum patulum. The structures of compounds 1-8 were characterized using spectroscopic analysis, calculations of nuclear magnetic resonance chemical shifts with DP4+ probability analysis, and electronic circular dichroism calculations. Compounds 2-4 exhibited immunosuppressive activities in an in vitro model of concanavalin A (Con A)-induced...

Drug-induced autoimmune-like hepatitis (DI-ALH) is a rare form of drug-induced liver injury (DILI) that mimics autoimmune hepatitis (AIH). Commonly implicated agents include minocycline, nitrofurantoin, and infliximab, though a wide range of other drugs has been reported. We report a 65-year-old woman with a history of suspected turmeric-induced liver injury six years prior who presented with nausea and painless jaundice two weeks after restarting the same turmeric supplement containing black...

Glomerulonephritis (GN), secondary to tuberculosis, is quite a rare condition. Here we report the case of membranous nephropathy, which completely resolved after the treatment of tuberculosis. A 42-year-old man presented to a nephrologist with burning micturition. His urine routine examination (RE) was suggestive of 2+ protein. His 24-hour urinary protein was 4.2 grams. Renal biopsy revealed membranous nephropathy. His autoimmune, hepatitis B, hepatitis C, and HIV workups were non-conclusive....

CONCLUSION: OS of patients diagnosed with incCCA was similar to HCC patients. These results add data to support transplantation of specific CCA patients as a reasonable treatment option.

Glycyrrhizin preparations (GLPS), derived from Glycyrrhiza uralensis (licorice), are widely used hepatoprotective agents in clinical practice. Their primary active components, 18α- and 18β-glycyrrhetinic acid (GA), are represented by formulations including diammonium glycyrrhizinate (DG), compound glycyrrhizin (CG), and magnesium isoglycyrrhizinate (MgIG). This review provides a comprehensive overview of the pharmacokinetic properties of GLPS and their multi-target pharmacological mechanisms,...