Immunoglobulin G4 (IgG4) Related Disease

Krati Chauhan, Phil A. Hart, Vaidehi R. Chowdhary

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized syndrome characterized by frequent elevation of serum Ig4 levels and typical pathology of the affected organs. Biopsy shows lymphoplasmacytic inflammation, frequent eosinophils, storiform fibrosis, obliterative phlebitis and abundant IgG4-positive staining plasma cells. Disease manifestations often respond dramatically to steroid therapy.

Original languageEnglish (US)
Title of host publicationChallenging Cases in Rheumatology and Diseases of the Immune System
PublisherSpringer New York
Pages73-88
Number of pages16
ISBN (Print)9781461450887, 146145087X, 9781461450870
DOIs
StatePublished - Mar 1 2014

Keywords

  • Aortitis
  • Autoimmune pancreatitis
  • IgG4
  • Orbital pseudolymphoma

ASJC Scopus subject areas

  • Medicine(all)

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  • Cite this

    Chauhan, K., Hart, P. A., & Chowdhary, V. R. (2014). Immunoglobulin G4 (IgG4) Related Disease. In Challenging Cases in Rheumatology and Diseases of the Immune System (pp. 73-88). Springer New York. https://doi.org/10.1007/978-1-4614-5088-7_5