Related Experiment Video
Updated: May 21, 2026

In Vivo Two-Color 2-Photon Imaging of Genetically-Tagged Reporter Cells in the Skin
Published on: July 11, 2019
IgG4-Related Fibrotic Diseases from an Immunological Perspective: Regulators out of Control?
Laura C Lighaam1, Rob C Aalberse, Theo Rispens
1Landsteiner Laboratory, Academic Medical Centre, University of Amsterdam, 1066 CX Amestrdam, The Netherlands.
Abstract:
Patients with autoimmune pancreatitis have a striking polyclonal elevation of total IgG4 in serum. This observation has been confirmed and extended to other fibrotic conditions (that are therefore called IgG4-related disease) but as yet remains unexplained. The affected tissue contains many IgG4-producing plasma cells embedded in a fibrotic matrix originating from activated mesenchymal (stellate) cells. We propose that the process results from an unusual interaction between two regulatory systems: the regulatory arm of the immune system (including Bregs) and the tissue repair regulatory components orchestrated by the activated stellate cell. This interaction results in ongoing mutual activation, generating TGFbeta, IL10, and vitamin D. This environment suppresses most immune reactions but stimulates the development of IgG4-producing plasma cells.
Related Concept Videos
Chronic Inflammation: Introduction
Inflammatory Bowel Disease III: Crohn's Disease
T Cell Types and Functions
Th1 cells stimulate dendritic cells to express necessary co-stimulatory molecules on their surfaces for...
Cirrhosis II: Pathophysiology
Introduction to Fibroblasts
Type IV Collagen of Basal Lamina
A type IV collagen molecule has six alpha chains which can exist in...

