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Journey to diagnosis: An unfinished exploration of IgG4-related sclerosing cholangitis.

World J Clin Cases

November 2024

Department of Infectious Diseases, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China.

IgG4-related sclerosing cholangitis (IgG4-SC) is an inflammatory disease that leads to bile duct stricture, characterized by the infiltration of IgG4-positive plasma cells into the bile duct wall, thickening of the bile duct wall, and narrowing of the lumen. The differential diagnosis of IgG4-SC mainly includes primary sclerosing cholangitis, cholangiocarcinoma, and pancreatic cancer. IgG4-SC is often associated with autoimmune pancreatitis and can be accurately diagnosed based on clinical diagnostic criteria.

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[Clinical characteristics and prognosis analysis of patients with IgG4-related hepatobiliary-pancreatic disease].

Zhonghua Gan Zang Bing Za Zhi

October 2024

Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Institute of Digestive Disease, Shanghai200001, China.

To explore and analyze the clinical features of patients with immunoglobulin (Ig)G4-related hepatobiliary-pancreatic disease and the independent factors affecting the prognosis of IgG4-related sclerosing cholangitis (IgG4-SC). The clinical data of 179 adult cases diagnosed with IgG4-related hepato-pancreato-biliary disease in the Division of Gastroenterology and Hepatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine from January 2011 to December 2022 were retrospectively analyzed. Patients were divided into three groups: isolated IgG4-SC, IgG4-SC/type 1 autoimmune pancreatitis(type 1 AIP), and isolated AIP according to the clinical manifestations.

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A 72-year-old man presented with several months of weakness, poor appetite, and depressed moods. Laboratory tests indicated central hypocortisolism, hypothyroidism and hypogonadism, and mild hyperprolactinemia. Imaging indicated a homogenously enhancing solid suprasellar mass inseparable from the hypothalamus and contiguous with a thickened proximal infundibulum.

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Case Report: IgG4-Related Disease Presenting With Isolated Hypophysitis.

AACE Clin Case Rep

July 2024

Division of Endocrinology and Metabolism, Jewish General Hospital, McGill University, Montreal, Quebec, Canada.

Article Synopsis
  • IgG4-related disease (IgG4-RD) is an immune condition that can affect multiple organs, including the pituitary gland, and can present as hypophysitis, which may mimic more severe issues like pituitary apoplexy.
  • A 49-year-old woman experienced symptoms such as abdominal pain and low cortisol levels, leading to imaging that revealed a pituitary macroadenoma; however, surgery revealed the condition to be IgG4-related hypophysitis, not a tumor.
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IgG4-related disease in the nervous system.

Pract Neurol

September 2024

Department of Neurology, Southmead Hospital, North Bristol NHS Trust, Bristol, UK

IgG4-related disease (IgG4-RD) is a recently described multisystemic disorder with a spectrum of manifestations that continue to be described. Nonetheless, there are recognised distinct patterns of disease. Neurological involvement is rare, particularly in isolation, but IgG4-RD may present with orbital disease, hypophysitis or pachymeningitis.

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