Immunoglobulin G4-Related Disease of the Intestine : A Clinicopathological Entity to Be Considered

Background and Objectives: Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized immune-mediated, systemic condition of unknown etiology, associated with fibroinflammatory lesions. Diagnosis is set in the presence of IgG4-positive plasma cell infiltration of the involved tissue and elevated serum IgG4 levels. However, approximately 30% of patients have normal serum IgG4 levels. IgG4-RD may affect several organs, including the pancreas, bile ducts, mesentery, retroperitoneum, and salivary glands, but the involvement of the gastrointestinal tract is uncommon. Materials and Methods: The case series of 4 patients with IgG4-RD involving the intestinal tract was observed in the period of 2017-2022. Colorectal and ileal biopsy specimens were stained with hematoxylin and eosin and immunohistochemical techniques using monoclonal antihuman IgG4 primary antibody. Diagnosis of IgG4-RD was based on the presence of >50 cells/ HPF and IgG4/IgG ratio >40 confirmed by two pathologists. Results: IgG4-RD was set in patients previously diagnosed as affected by Crohn's disease. Conclusions: Systematic IgG4 immunohistochemical staining should be considered in the diagnostic workup of patients with gastrointestinal strictures, mimicking Crohn's disease. The exact prevalence of the condition is likely more frequent than reported and should be defined by a large series of consecutive patients.

Medienart:

E-Artikel

Erscheinungsjahr:

2023

Erschienen:

2023

Enthalten in:

Zur Gesamtaufnahme - volume:60

Enthalten in:

Medicina (Kaunas, Lithuania) - 60(2023), 1 vom: 28. Dez.

Sprache:

Englisch

Beteiligte Personen:

Vernia, Filippo [VerfasserIn]
Cirella, Laura [VerfasserIn]
Calvisi, Giuseppe [VerfasserIn]
Viscido, Angelo [VerfasserIn]
Latella, Giovanni [VerfasserIn]

Links:

Volltext

Themen:

Antibodies, Monoclonal
Case Reports
Crohn’s disease
IgG4-RD
Immunoglobulin G
Immunoglobulin G4-related disease
Inflammatory bowel diseases
Intestinal strictures

Anmerkungen:

Date Completed 24.01.2024

Date Revised 29.01.2024

published: Electronic

Citation Status MEDLINE

doi:

10.3390/medicina60010057

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM367468042