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[IgG4-associated diseases : Essentials for pathologists and radiologists].

A Tannapfel1

  • 1Institut für Pathologie, Ruhr-Universität Bochum, Bürkle-de-la-Camp Platz 1, 44789, Bochum, Deutschland. andrea.tannapfel@rub.de.

Der Radiologe
|October 19, 2016
PubMed
Summary

Immunoglobulin G4-associated (IgG4) disease is a systemic condition affecting multiple organs. Prompt response to glucocorticoids highlights its importance for pathologists and radiologists.

Keywords:
BiopsyLymphoplasmacellular inflammationMultisystemic diseaseObliterative phlebitisStoriform fibrosis

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Area of Science:

  • Immunology
  • Pathology
  • Radiology

Background:

  • Immunoglobulin G4-associated (IgG4) diseases are systemic, multiorgan conditions with diverse clinical presentations.
  • A common morphological feature across IgG4-associated diseases is lymphoplasmacellular inflammation, characterized by numerous IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis.

Purpose of the Study:

  • To highlight the systemic nature and diagnostic considerations of IgG4-associated diseases.
  • To emphasize the need for awareness among pathologists and radiologists regarding this emerging multisystemic disease.

Main Methods:

  • Review of morphological correlates in IgG4-associated diseases.
  • Clinical observation of treatment response in IgG4-associated diseases.

Main Results:

  • IgG4-associated diseases exhibit a consistent set of histopathological findings, including lymphoplasmacellular inflammation, obliterative phlebitis, and storiform fibrosis.
  • Glucocorticoids typically induce a rapid clinical improvement, suggesting a role in managing these conditions.

Conclusions:

  • IgG4-associated diseases are multisystemic conditions requiring consideration in differential diagnoses.
  • Pathologists and radiologists should be aware of IgG4-associated diseases due to their variable presentation and responsiveness to glucocorticoid therapy.