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

Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease primarily affecting the intrahepatic small bile ducts. Although PBC is mainly a hepatobiliary disease, renal abnormalities have been reported in a subset of patients and should be regarded as uncommon but clinically relevant extrahepatic manifestations. Reported glomerular lesions in patients with PBC include membranous nephropathy, immunoglobulin A nephropathy, crescentic glomerulonephritis, and minimal change...

CONCLUSIONS: In this retrospective cohort of patients with ulcerative colitis and PSC undergoing IPAA, usage of concomitant antibiotics or immunosuppression was low. However, use of concomitant antibiotics or immunosuppression may decrease the burden of inflammatory conditions of the pouch in these high-risk patients.

Postpartum autoimmune hepatitis (AIH) is a rare but serious condition triggered by immune rebound after delivery. Diagnosis is typically supported by elevated immunoglobulins and positive autoimmune markers; however, seronegative presentations remain diagnostically challenging. We present the fourth reported worldwide case of seronegative postpartum AIH in a 36-year-old woman who developed jaundice, pruritus, pale stools, and dark urine 4 months after delivery. Laboratory evaluation revealed...

Disseminated histoplasmosis (DH) is a common opportunistic mycosis in endemic regions, yet it remains underreported in pediatric patients with primary immunodeficiency or acquired immunosuppression. We present the clinical progression and fatal outcome of DH in a 12-year-old female from southeastern Honduras with an established diagnosis of Ataxia-Telangiectasia (AT) and Autoimmune hepatitis (AIH) managed with daily prednisone (1 mg/kg/day) and azathioprine (2 mg/kg/day), presented with fever,...

CONCLUSIONS: An empirical gradual corticosteroid tapering strategy developed with reference to Japanese AIH management may contribute to relapse prevention and facilitate ICI rechallenge in patients with ICI-induced liver injury.

Primary sclerosing cholangitis (PSC) is a rare cause of pediatric cholestasis and may exceptionally present as a focal mass-forming lesion. We report the case of a 9-year-old boy presenting with progressive obstructive jaundice in whom magnetic resonance cholangiopancreatography revealed a solid lesion at the hepatic duct confluence, highly suspicious for malignancy. Given persistent biliary obstruction and the inability to reliably exclude cholangiocarcinoma using noninvasive or endoscopic...

CONCLUSIONS: Given its diverse etiologies, SSC requires a structured evaluation that integrates exposure history, laboratory investigations, magnetic resonance cholangiopancreatography (MRCP)/endoscopic retrograde cholangiopancreatography (ERCP) patterns, and histology for diagnosis. Etiology-specific therapy may slow progression, but early transplant referral is critical for rapidly progressive phenotypes.

Acute systemic infection may cause hepatic, hematological and radiologic abnormalities that closely mimic decompensated liver cirrhosis. Vibrio cholerae causes cholera, which does not typically imply hepatic involvement; however, in severe or complicated cases, there may be dysfunction of various organs, cholestatic hepatitis, coagulopathy, and false radiographic findings. In addition, acute infections can serve as immunological triggers for autoimmune hepatitis (AIH), an infrequent yet...

CONCLUSION: This integrative analysis identified six candidate host targets associated with high-risk PBC. CCL2 showed the most convergent evidence within the discovery framework and may represent a chemokine-related hepatic microenvironment-associated candidate target. In contrast, CD44, FOS, and CDKN1A showed supportive expression patterns in the GSE119600 blood cohort. These findings represent exploratory candidate prioritization, and further validation in independent liver tissue cohorts and...

Chronic liver diseases of diverse etiologies, along with primary liver cancers, represent major global health burdens with limited curative options. CD8^(+) T-cell exhaustion is a central barrier to effective immune control in these pathological settings. However, precursor exhausted CD8^(+) T cells (Tpex), a stem-like subset with self-renewal and proliferative potential, retain the ability to generate effector-like progeny and sustain long-term immune surveillance. In this review, we first...

Background/Objectives: Selecting the optimal biliary reconstruction technique for liver transplantation (LT) in primary sclerosing cholangitis (PSC) remains challenging. Many centers have historically favored Roux-en-Y hepaticojejunostomy (RYHJ) to bypass potentially diseased recipient ducts, whereas duct-to-duct (D-D) anastomosis preserves endoscopic access and decreases cholangitis rates. We evaluated outcomes during an institutional, anatomy-driven transition toward D-D reconstruction....

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CONCLUSION: IgG4-RD with initial diarrhea and jaundice is easily misdiagnosed as cholangiocarcinoma or primary sclerosing cholangitis. Markedly elevated serum IgG4 combined with characteristic multi-organ imaging changes can strongly support a clinical suspicion of IgG4-RD, but cannot independently establish a definitive diagnosis without histopathological evidence. The gold-standard definite diagnosis requires integrated fulfillment of clinical, serological, radiological and pathological...

Idiosyncratic drug-induced liver injury (iDILI) is not a uniform disease but rather includes a variety of types based on immune, autoimmune, and clinical considerations. This review attempts to close information gaps regarding the role of immunity and autoimmunity involved in the different iDILI disease types. The analysis of the current literature focusing on iDILI reveals compelling evidence of a pivotal role of immunity or autoimmunity in various types of iDILI. Among the autoimmune-triggered...

Aim: Primary biliary cholangitis (PBC) is a rare liver disease associated with high morbidity. This study assessed the burden of fatigue and/or pruritus among patients with PBC in the US. Materials & methods: This retrospective study used IQVIA PharMetrics^(®) Plus data (2016-2022). Patients with PBC and fatigue and/or pruritus were selected as cases. Controls were patients with PBC (no fatigue nor pruritus), matched 1:1 to cases by key characteristics. The index date for cases was a random...

BACKGROUND: Gallbladder carcinoma is an uncommon but highly lethal malignancy that is frequently diagnosed at an advanced stage. Among proposed precursor lesions, true neoplastic gallbladder polyps (particularly adenomas and intracholecystic papillary neoplasms) have received increasing attention as potential steps in an adenoma-carcinoma sequence, analogous to colorectal tumorigenesis [CA Cancer J Clin. 2021;71(3):209-49, Br J Radiol. 2022;95(1137):20220152, Eur Radiol. 2022;32(5):3358-68, and...

CONCLUSION: The ET model constructed in this study enables precise prognosis prediction for PBC patients. After SHAP analysis, it demonstrates good interpretability. The key prognostic features identified by the model provide quantitative evidence for clinically assessing disease severity in PBC patients and offer data support for developing individualized clinical intervention plans.

Background & aim: Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease that can lead to increased morbidity and mortality. This study described real-world treatment patterns and clinical outcomes by line of treatment among patients with PBC in the US. Materials & methods: Adults (≥18 years) diagnosed with PBC on or after 1 January 17 were identified in the IQVIA PharMetrics^(®) Plus database and grouped into newly diagnosed, first-line (1L) and second-line or more (2L+)...

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Primary sclerosing cholangitis (PSC) is a chronic autoimmune cholestatic liver disease characterized by progressive inflammation and fibrosis, but the role of neutrophils in PSC remains poorly understood. To define neutrophil states in the PSC liver microenvironment, we performed single-cell RNA sequencing of leukocytes isolated from liver perfusate obtained from healthy donor grafts and explanted PSC livers. We identified a marked expansion of transcriptionally diverse neutrophil subclusters in...