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

Cirrhosis is a known cause of secondary IgA nephropathy (IgAN), often presenting with a membranoproliferative glomerulonephritis (MPGN) pattern and poor renal outcomes. We report a case of IgAN with an MPGN pattern associated with autoimmune hepatitis (AIH) in a 74-year-old woman. Although the patient had liver cirrhosis, her AIH was in clinical remission following prior corticosteroid therapy. She presented with nephrotic-range proteinuria and lower leg edema. Renal biopsy confirmed IgAN with...

Hereditary hemochromatosis is a disorder of iron metabolism characterized by increased intestinal iron absorption and progressive parenchymal iron deposition, most commonly associated with homozygous mutations in the HFE gene. In contrast, heterozygous carriers of HFE mutations are generally considered to have low clinical penetrance and are not expected to develop clinically significant iron overload or advanced liver disease. However, iron homeostasis may be substantially altered in the...

Intraductal papillary neoplasm of the bile duct (IPNB) is a biliary epithelial tumor that represents a recognized precursor lesion of cholangiocarcinoma. We report the case of a 60-year-old woman who presented to the emergency department with acute abdominal pain. A CT scan performed for suspected nephrolithiasis incidentally revealed an irregular hypodense lesion in segment VI of the liver with associated intrahepatic bile duct dilatation. Further evaluation with MRI and magnetic resonance...

CONCLUSIONS: This case highlights the complexity of managing pediatric patients with multiple autoimmune diseases. The necessary combination of immunosuppressive therapies may lead to significant adverse effects and further complicate disease progression. Moreover, psychological components play a crucial role in treatment compliance and therapeutic success, emphasizing the need for an integrated approach that includes specialized psychological support.

CONCLUSION: Cystic dilatation appears to be a clinically relevant prognostic feature in patients with primary sclerosing cholangitis, however, confirmation in larger studies is warranted.

CONCLUSIONS: AIH patients with concomitant HS had worse outcomes than those without HS; whereas HS did not influence the clinical outcomes in patients with PBC. Future research evaluating the impact of HS on PSC and overlap syndrome is much needed.

CONCLUSION: Pediatric IBD-associated AILDs represent a heterogeneous and high-risk group of disorders. Multimodal diagnostic strategies and immunosuppressive therapy are central to management; however, effective treatments for PSC remain lacking, highlighting major unmet clinical needs.

CONCLUSIONS: PBC is consistently associated with higher waitlist mortality risk compared with PSC, AIH, and ALD, highlighting potential under-prioritization of this condition for liver transplant. Allocation policies may consider disease-specific exceptions or prognostic models tailored to AILDs to reduce waitlist mortality disparities.

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Emerging evidence has highlighted the gut-brain axis as a critical mediator in the pathogenesis of both major depressive disorder (MDD) and autoimmune hepatitis (AIH), where systemic inflammation and gut barrier dysfunction play pivotal roles. This study investigated the therapeutic potential of avanafil (AVA), a selective phosphodiesterase-5 inhibitor (PDE5I), in a lipopolysaccharide (LPS)-induced rat model that mimics inflammation-driven MDD and AIH. LPS administration significantly impaired...

CONCLUSIONS: While musculoskeletal manifestations are common, fewer than 5% of pIBD patients experience ophthalmological, dermatological, and liver IM-EIMs. The small number of studies resulted in significant methodological and statistical heterogeneity. Multicenter collaborative efforts are needed to systematically describe the epidemiology of IM-EIMs in pIBD.

Labetalol is widely prescribed as a first-line antihypertensive in pregnancy; however, rare but potentially severe idiosyncratic hepatotoxicity has been reported, with a disproportionately strong reporting signal for liver injury relative to other β-blockers. In this report, a fatal case is described in which mild initial symptoms rapidly progressed to fulminant hepatocellular injury and acute liver failure within days, underscoring the importance of early recognition of drug-induced liver...

Wilson's disease (WD) is an autosomal recessive disorder caused by mutations in the ATP7B gene, leading to impaired copper metabolism. Its clinical presentation is heterogeneous and may mimic autoimmune hepatitis (AIH), making timely diagnosis challenging, particularly in adolescents with severe liver disease of unknown origin. A retrospective descriptive study was conducted based on the medical record review of a patient diagnosed with WD and treated at a university referral hospital. The study...

CONCLUSION: SLE and AIH represent overlapping entities along a unified autoimmune spectrum, driven by shared genetic susceptibility, convergent immune dysregulation, and microbial influences. This review advances an integrated immunological framework that bridges systemic and organ-specific autoimmunity, underscores the pivotal role of innate immunity and gut-liver crosstalk, and provides a mechanistic rationale for cross-disease therapeutic strategies targeting these common pathways.

Dendriform pulmonary ossification (DPO) is a rare condition characterised by branching bone formation within the lung parenchyma, often associated with fibrotic interstitial lung disease (ILD). We report an asymptomatic man in his 80s with primary biliary cholangitis who presented with bilateral lower-lobe fibrosis and subpleural micronodules on high-resolution CT, initially suggesting granulomatous ILD. Transbronchial lung cryobiopsy revealed fibrosis with intra-alveolar ossification and...

CONCLUSION: This case illustrates a multifactorial liver injury wherein amoxicillin-clavulanate served as the primary hepatotoxin, with possible warfarin-related hepatotoxicity and coagulopathy superimposed on latent PBC. It emphasizes the need for vigilance when prescribing interacting with or hepatotoxic agents to patients on chronic anticoagulation. The case further demonstrates the supportive-but not definitive-role of RUCAM in polypharmacy-related DILI assessment, reinforcing the primacy of...

Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, yet the cellular crosstalk driving periductal fibrosis remains poorly defined. This study applied a multi-omics approach integrating spatial transcriptomics, RNA-seq, and proteomics to characterize fibrotic periductal regions and their cell-cell communications. Macrophages (MP) subsets, including monocyte-drived-(Mo)MP and...

CONCLUSIONS: Gli1 demarcates a subpopulation of fibroblasts that play a key role in the wound healing process combining portal fibrosis and ductular reaction notably in biliary diseases.

CONCLUSION: This study identifies a high prevalence of NCPF in early PBC. Its association with greater biochemical activity and specific vascular histopathological alterations suggests that the presence of NCPF may delineate a more active PBC phenotype, offering potential value for early risk assessment.

CONCLUSIONS: In pediatric patients presenting with GD-associated liver dysfunction, AIH should be considered as a potential comorbidity, warranting vigilance in patients with hyperthyroidism and elevated transaminase levels.