Key Clinical Message: This case highlights the importance of a definite diagnosis of an IgG4-related chronic sclerosing duodenitis based on histological and radiological findings to rule out any malignancy in the mass. While dealing with patients having concentric duodenal thickening resulting in stricture formation, one should think of inflammatory etiology as well. IgG4-related disease is one of these inflammatory disorders where we see soft tissue thickening without a large mass or any associated lymphadenopathy as in our case.

Abstract: Immunoglobulin G4-related disease (IgG4-RD) is distinguished as an infiltration of IgG-4-positive plasmacytes involving inflammatory lesions across multiple organs which is accompanied by raised IgG4 levels in the serum. Several inflammatory disorders are recognized as part of the IgG4-RD family based on shared histopathological features, which include Mikulicz's disease, chronic sclerosing sialadenitis, or Riedel's thyroiditis. Our case highlights a distinctive presentation of IgG4-related diseases; a 58-year-old man presenting with duodenal stricture highly suspicious of a duodenal mass/ampullary mass later found to be due to IgG4-related sclerosing duodenitis with negative malignancy on biopsy. We present the diagnostic challenges faced and relevant findings noted.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11130227PMC
http://dx.doi.org/10.1002/ccr3.8980DOI Listing

Publication Analysis

Top Keywords

chronic sclerosing
12
duodenal stricture
8
igg4-related chronic
8
case highlights
8
sclerosing duodenitis
8
inflammatory disorders
8
igg4-related
5
duodenal
4
stricture secondary
4
secondary igg4-related
4

Similar Publications

.

Expert Rev Gastroenterol Hepatol

January 2025

Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of California San Diego, San Diego, CA.

Introduction: The gut microbiota has a complex relationship with the human host and is key to maintaining health. Disruption of the healthy diverse gut microbial milieu plays an important role in the pathogenesis of several diseases including infection (CDI), inflammatory bowel disease, irritable bowel syndrome, alcohol-related liver disease and metabolic-dysfunction associated steatotic liver disease (MASLD). Fecal microbiota transplantation (FMT) is highly effective in treating CDI, though its utility in other diseases is still being explored.

View Article and Find Full Text PDF

Sclerosing Mesenteritis Presenting as Intestinal Occlusion: A Case Report.

Cureus

December 2024

Esophagogastric Surgery, Unidade Local de Saúde (ULS) de Viseu Dão-Lafões, Viseu, PRT.

Article Synopsis
  • Sclerosing mesenteritis is a rare chronic condition that affects the mesenteric fat, involving inflammation and fibrosis without a clear cause, leading to variable symptoms, most commonly abdominal pain.
  • Its diagnosis primarily relies on histological analysis, even though incidental findings have increased due to widespread CT imaging.
  • The prognosis is generally good, but misdiagnosis is common, highlighting the need to consider it in cases of vague abdominal pain to prevent unnecessary treatments, though surgery might be required for severe cases like intestinal blockage.
View Article and Find Full Text PDF

Gut Microbiome and Bile Acid Interactions: Mechanistic Implications for Cholangiocarcinoma Development, Immune Resistance, and Therapy.

Am J Pathol

December 2024

Department of Microbiology and Immunology, Virginia Commonwealth University and Richmond VA Medical Center, Richmond, Virginia; Stravitz-Sanyal Institute for Liver Disease and Metabolic Health, School of Medicine, Virginia Commonwealth University, Richmond, Virginia. Electronic address:

Cholangiocarcinoma (CCA) is a rare but highly malignant carcinoma of bile duct epithelial cells with a poor prognosis. The major risk factors of CCA carcinogenesis and progression are cholestatic liver diseases. The key feature of primary sclerosing cholangitis and primary biliary cholangitis is chronic cholestasis, which means a slowdown of hepatocyte secretion of biliary lipids and metabolites into bile as well as a slowdown of enterohepatic circulation (bile acid recirculation) of bile acids with dysbiosis of the gut microbiome, which was shown to lead to enterohepatic recirculation and an increase of toxic secondary bile acids.

View Article and Find Full Text PDF

A novel multi-organ male model of alcohol-induced acute-on-chronic liver failure reveals NET-mediated hepatocellular death which is prevented by RIPK3 inhibition.

Cell Mol Gastroenterol Hepatol

December 2024

Department of Medicine, Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA; Broad Institute, Cambridge, MA. Electronic address:

Background And Aims: Alcohol abuse is the most frequent precipitating factor of acute-on-chronic liver failure (ACLF). We aimed at developing an alcohol-induced ACLF model and dissecting its underlying molecular mechanisms.

Methods: ACLF was triggered by a single alcohol binge (5g/Kg) in a bile duct ligation (BDL) liver fibrosis murine model.

View Article and Find Full Text PDF

Primary chronic osteomyelitis (PCO) is a rare non-bacterial autoinflammatory severe disease that most often affects children aged 7 to 12 years and is much less common in adults. The most common areas of lesion are long tubular bones, however, any bones of the skeleton, including the lower jaw, can be affected. The clinical picture of this disease is complex and similar to many tumor and tumor-like bone lesions, which often leads to a significant delay in making a correct diagnosis.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!