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Cholecystectomy Is Linked to Worse Clinical Outcomes in Primary Sclerosing Cholangitis.

Recent findings have suggested that gallbladder-derived retinoic acid signaling plays a crucial role in the regeneration of damaged intrahepatic biliary ducts. This retrospective cohort study analyzed the clinical records of 20 patients with primary sclerosing cholangitis (PSC) treated at our hospital between 2013 and 2024. We investigated the clinical implications of gallbladder removal in patients with PSC, a progressive cholangiopathy with limited therapeutic options. We retrospectively analyzed the data of patients with PSC and compared patients with and without prior cholecystectomy to as…

Correction: Biliary stents reshape the bile microbiome in the absence of cholangitis.

[This corrects the article DOI: 10.1055/a-2733-3468.].

Immunological and fibrotic profiling in the NOD.c3c4 murine model of autoimmune cholangitis.

Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disease characterized by intrahepatic bile duct destruction, progressive cholestasis, and fibrosis, accompanied by elevated anti-mitochondrial antibodies (AMAs) and IgM. While multiple mouse models have been developed to study PBC pathogenesis, no single model fully recapitulates all aspects of human disease. Among these, the NOD.c3c4 mouse model, generated by incorporating B10 and B6 insulin-dependent diabetes (Idd) loci into NOD mice, uniquely develops PBC-like features without progressing to type-1 diabetes. NOD.c3c4 exhibit PD…

The role of HLA-G in primary biliary cholangitis and response to therapy.

INTRODUCTION: Primary biliary cholangitis (PBC) is a rare autoimmune liver disease involving bile duct damage and fibrosis. This study explores the role of HLA-G, an immunomodulatory molecule crucial for immune tolerance, in PBC pathogenesis and treatment. METHODS: A cohort of 166 PBC patients from Sardinia was compared to 180 healthy controls and 205 autoimmune hepatitis type 1 (AIH-1) patients. Plasma soluble HLA-G (sHLA-G) levels, HLA-G alleles, and 3'UTR haplotypes were analyzed alongside clinical data, including therapy response to ursodeoxycholic acid. RESULTS: The UTR-1 haplotype was …

An update on novel investigational agents for the treatment of primary biliary cholangitis.

INTRODUCTION: Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by progressive bile duct destruction that can lead to liver cirrhosis and liver failure. While ursodeoxycholic acid (UDCA) remains the first-line treatment, up to 40% of patients show an inadequate response. In such cases, second-line therapies are explored. Obeticholic acid (OCA), a farnesoid X receptor agonist, was initially approved but recently lost its marketing authorization in the EU due to an unfavorable risk-benefit balance. Fibrates, particularly bezafibrate and fenofibrate, have shown…

Methylene Blue for Refractory Shock Due to Acute Pancreatitis and Cholangitis.

Methylene blue is emerging as a promising adjunctive therapy for refractory septic shock, enabling reduced vasopressor use and fewer intensive care and hospital days. We present the case of a 78-year-old woman with acute pancreatitis and cholangitis who was initially treated with antibiotics, IV fluids, and endoscopic retrograde cholangiopancreatography but had persistent shock with vasopressor requirements. She was then treated with methylene as a 1 mg/kg bolus, followed by another 1 mg/kg infusion over six hours, to reduce overall pressor requirements. She improved significantly within hours…

Lemmel Syndrome Presenting with Transient Cholangitis and Bacteremia: A Case Report.

Lemmel syndrome is a rare disease characterized by mechanical obstruction of the bile duct secondary to a periampullary duodenal diverticulu … f duodenal diverticula without any evidence of an obstructive cause, Lemmel syndrome with transient cholangitis was considered the most likely diagnosis. Unlike most published reports of Lemmel syndrome presenting with persistent obstructive jaundice, our case is distinctive in its transient presentation complicated by bacteremia, which posed diagnostic chall …

Acute cholecystitis and acute cholangitis: the radiologist's role in diagnosis and management.

Acute cholecystitis (AC) and acute cholangitis (ACh) are common biliary conditions that can range in clinical severity from mild discomfort to life-threatening complications. The Tokyo Guidelines (TG) recommend a diagnostic approach that integrates clinical signs, laboratory findings, and imaging features to improve diagnostic accuracy. However, diagnosis might be difficult given overlapping clinical symptoms and the different imaging presentations of different disorders. A coordinated multidisciplinary approach is necessary for effective management, in which radiologists are pivotal in both d…

The immunological landscape of primary biliary cholangitis: Mechanisms and therapeutic prospects.

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease characterized by the progressive destruction of intrahepatic bile ducts, leading to fibrosis, and potentially cirrhosis. PBC has been considered a prototypical autoimmune condition, given the presence of specific autoantibodies and the immune response against well-defined mitochondrial autoantigens. Further evidence supports the interaction of immunogenetic and environmental factors in the etiology of PBC. An immunological attack on biliary epithelial cells with secondary failure of biliary transporters, eg, the anion exc…

Primary sclerosing cholangitis: a narrative review of diagnostic and prognostic biomarkers.

BACKGROUND AND OBJECTIVE: Primary sclerosing cholangitis (PSC) is an autoimmune biliary fibrosing disease characterized by inflammation and injury of the intra- and/or extrahepatic bile ducts. The pathogenesis of PSC is poorly understood but is believed to be multifactorial, involving genetic predisposition, immunological dysregulation, and environmental influences. These may include disturbances in the gut-liver axis such as immune dysfunction in the colon and liver, alterations in the fecal and biliary microbiome, conjugation of bile acids into toxic species, and compromised intestinal epith…

Secondary HIV Cholangiopathy as a Cause of Acute Cholangitis: A Case Report.

HIV-associated cholangiopathy (HAC) is a rare but clinically important hepatobiliary complication occurring in patients with advanced immuno … italization, a complex distal common bile duct (CBD) stricture was identified, accompanied by acute cholangitis, obstructive pancreatitis, and acute kidney injury (AKI) on a background of chronic kidney disease (CKD). The patient underwent endoscopic retrograde cholangiopancreatography (ERCP) with biliary stent placement, resulting in clinical improvement. HI …

Первичный билярийный чолангит у пациента с множественным склерозом: отчет по делу.

Множественный склероз (MS) — хроническое аутоиммунное состояние, характеризующееся демиэлизацией центральной нервной системы. В литературе редко описывается ее сосуществование с первичным холангитом (КМС), еще одним аутоиммунным заболеванием. Так обстоит дело с 35-летней пациенткой Эмирати с историей ПМС, у которой были неустойчивая походка и неврологические симптомы. Неврологический осмотр выявил дисметрию, широкую походку и резкие рефлексы. Результаты МРТ подтвердили наличие MS, и ее лечили метилпреднизолоном и лечением болезней. MS и КМС делятся иммунной дисрегуляцией, включая генетические

Совпадение в аннотации оригинала
Multiple sclerosis (MS) is a chronic autoimmune condition characterized by central nervous system demyelination. Its coexistence with primary biliary cholangitis (PBC), another autoimmune disease, is rarely described in the literature. This is the case of a 35-year-old Emirati female patient with a history of PBC who presented with unsteady gait and neurolog…

Recurrent cholangitis and bacteraemia due to Edwardsiella tarda: a case report.

Edwardsiella tarda is typically isolated from aquatic environments. It rarely causes infections in humans. Edwardsiella tarda infections in humans result from the consumption of infected or contaminated food. Here, we present a case of recurrent cholangitis and bacteraemia associated with E. tarda. An 82-year-old man with no history of seafood inoculation was admitted to our hospital because of difficulty in moving his body. The patient was diagnosed with cholangitis, and the blood culture revealed the presence of E. tarda. The patient underwent bile duct stenting and received antibiotic thera…

Increased risk of fragility fractures in patients with primary biliary cholangitis.

Large-scale studies on the risk of fragility fractures in patients with primary biliary cholangitis (PBC) are limited due to low incidence. We aimed to investigate whether PBC is associated with fragility fractures using real-world nationwide data. The Korean National Health Insurance Service claims data from 2007 to 2020 were analyzed in this population-based cohort study. Patients with PBC (n = 4951) were matched with controls (n = 19 793) using a 1:4 ratio based on age, sex, and follow-up duration. The primary outcome was fragility fracture, which comprised fractures of the vertebra, hip, d…

Fecal microbiota transplantation as a preventive treatment for recurrent acute cholangitis.

BACKGROUND: Recurrent acute cholangitis (RAC) is a relatively uncommon entity that presents significant management difficulties. We present the case of a patient with RAC in whom the number of episodes was reduced after a novel therapeutic procedure. CASE REPORT: A 93-year-old male who in June 2019 was admitted for chills without fever, shivering, epigastric abdominal pain and moderate jaundice. Both abdominal ultrasound and CT scan showed intrahepatic and extrahepatic duct dilatation up to the papilla with no evidence of mass at that level. Endoscopic retrograde cholangiopancreatography (ERC…

The potential of volatile organic compounds to diagnose primary sclerosing cholangitis.

BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive inflammation and fibrosis of the bile ducts. PSC is a complex disease of largely unknown aetiology that is strongly associated with inflammatory bowel disease (IBD). Diagnosis, especially at an early stage, is difficult and to date there is no diagnostic biomarker. The present study aimed to assess the diagnostic potential of volatile organic compounds (VOCs) in exhaled breath to detect (early) PSC in an IBD population. METHODS: Breath samples were obtained from 16 patie…

Corrigendum to "The treatment of primary biliary cholangitis: from shadow to light".

[This corrects the article DOI: 10.1177/17562848241265782.].

The phenotypic landscape of primary biliary cholangitis and autoimmune hepatitis variants.

Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) stand as distinct diseases, yet occasionally intertwine with overlapping features, posing diagnostic and management challenges. This recognition traces back to the 1970s, with initial case reports highlighting this complexity. Diagnostic scoring systems like IAIHG and simplified criteria for AIH were introduced but are inherently limited in diagnosing variant syndromes. The so-called Paris criteria offer a diagnostic framework with high sensitivity and specificity for variant syndromes, although disagreements among international …