Spectrum of disorders associated with elevated serum IgG4 levels encountered in clinical practice

Jay H Ryu, Ryohei Horie, Hiroshi Sekiguchi, Tobias D Peikert, Eunhee S. Yi

Research output: Contribution to journalArticle

63 Citations (Scopus)

Abstract

IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8) had an elevated serum IgG4 level (140mg/dL). IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4) had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

Original languageEnglish (US)
Article number232960
JournalInternational Journal of Rheumatology
Volume2012
DOIs
StatePublished - 2012

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Immunoglobulin G
Serum
Biliary Tract Diseases
Systemic Vasculitis
Pancreatic Diseases
Clinical Laboratory Techniques
Lung Diseases
Liver Diseases

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Spectrum of disorders associated with elevated serum IgG4 levels encountered in clinical practice. / Ryu, Jay H; Horie, Ryohei; Sekiguchi, Hiroshi; Peikert, Tobias D; Yi, Eunhee S.

In: International Journal of Rheumatology, Vol. 2012, 232960, 2012.

Research output: Contribution to journalArticle

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