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Related Concept Videos

Inflammatory Bowel Disease III: Crohn's Disease01:25

Inflammatory Bowel Disease III: Crohn's Disease

Crohn’s disease is a chronic, relapsing form of inflammatory bowel disease characterized by segmental, transmural inflammation that can affect any part of the gastrointestinal tract. Its pathogenesis arises from a combination of genetic susceptibility, environmental exposures, epithelial barrier dysfunction, and immune dysregulation. Together, these factors lead to an exaggerated immune response against components of the gut microbiome.Genetic and Environmental InfluencesMultiple genetic...
Inflammatory Bowel Disease II: Crohn's Disease01:30

Inflammatory Bowel Disease II: Crohn's Disease

Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by transmural...
Inflammatory Bowel Disease I: Ulcerative Colitis01:27

Inflammatory Bowel Disease I: Ulcerative Colitis

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The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
Graves' Disease I: Introduction01:28

Graves' Disease I: Introduction

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Inflammatory Bowel Disease IV: Clinical Manifestations

Inflammatory bowel disease (IBD) encompasses two major chronic disorders—ulcerative colitis and Crohn’s disease—each characterized by relapsing episodes of gastrointestinal inflammation. Although they share certain clinical features, their patterns of involvement and manifestations differ in ways that aid diagnosis and guide management.Ulcerative ColitisUlcerative colitis is limited to the colon and rectum and involves continuous inflammation of the mucosal layer. The disease course is marked...
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Inflammatory Bowel Disease I: Introduction

Inflammatory bowel disease is a group of chronic disorders marked by recurrent inflammation of the gastrointestinal tract due to an abnormal immune response against gut microflora. This leads to tissue damage. The two main forms are Crohn’s disease and ulcerative colitis.Crohn’s DiseaseCrohn’s disease is a relapsing inflammatory disorder that can affect any part of the GI tract, from the mouth to the anus. It involves all layers of the bowel wall (transmural) and shows “skip lesions” in which...

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Related Experiment Video

Updated: May 22, 2026

Dioscin Mediated IgA Nephropathy Alleviation by Inhibiting B Cell Activation In Vivo and Decreasing Galactose-Deficient IgA1 Production In Vitro
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[IgG4-related disease].

Yasuharu Sato1, Tadashi Yoshino

  • 1Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan. satou-y@cc.okayama-u.ac.jp

Rinsho Byori. the Japanese Journal of Clinical Pathology
|May 10, 2012
PubMed
Summary

Immunoglobulin G4-related disease (IgG4-RD) presents with characteristic lesions and elevated IgG4+ plasma cells. Laboratory findings are crucial for differentiating IgG4-RD from hyper IL-6 syndromes, which share some histological features.

Area of Science:

  • Immunology
  • Pathology
  • Rheumatology

Background:

  • Immunoglobulin G4-related disease (IgG4-RD) is a systemic syndrome characterized by lymphoplasmacytic infiltrates and sclerosis, often affecting exocrine tissues.
  • Ocular adnexal IgG4-RD typically involves bilateral lacrimal glands, with obliterative phlebitis being rare. Malignant lymphomas can arise from ocular adnexal IgG4-RD.
  • Hyper IL-6 syndromes, including multicentric Castleman's disease and rheumatoid arthritis, share histological criteria with IgG4-RD, complicating diagnosis via immunohistochemistry alone.

Purpose of the Study:

  • To highlight the diagnostic challenges in differentiating IgG4-related disease from hyper IL-6 syndromes.
  • To emphasize the critical role of laboratory findings in distinguishing these conditions.

Main Methods:

  • Review of clinical and laboratory characteristics of IgG4-related disease and hyper IL-6 syndromes.

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  • Comparison of diagnostic criteria, focusing on immunohistochemical staining and laboratory markers.

    • Main Results:

    • Immunohistochemical staining alone is insufficient for differential diagnosis between IgG4-RD and hyper IL-6 syndromes.
    • Hyper IL-6 syndromes exhibit elevated CRP, polyclonal hypergammaglobulinemia, anemia, and hypoalbuminemia.
    • IgG4-RD is not characterized by elevated serum IgA, IgM, or CRP levels, distinguishing it from hyper IL-6 syndromes.

    Conclusions:

    • Laboratory examination is essential for the accurate differential diagnosis of IgG4-related disease and hyper IL-6 syndromes.
    • Distinct laboratory profiles aid in distinguishing these conditions despite overlapping histological features.