Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2012:2012:602809.
doi: 10.1155/2012/602809. Epub 2012 Aug 26.

Pathologies Associated with Serum IgG4 Elevation

Mikael Ebbo  1 , Aurélie Grados, Emmanuelle Bernit, Frederic Vély, José Boucraut, Jean-Robert Harlé, Laurent Daniel, Nicolas Schleinitz
Affiliations

Pathologies Associated with Serum IgG4 Elevation

Mikael Ebbo et al. Int J Rheumatol. 2012.

Abstract

Statement of Purpose. IgG4-related disease (IgG4-RD) is usually associated to an increase of serum IgG4 levels. However other conditions have also been associated to high serum IgG4 levels. Methods. All IgG subclasses analyses performed in our hospital over a one-year period were analyzed. When IgG4 level were over 1.35 g/L, the patient's clinical observation was analyzed and both final diagnosis and reason leading to IgG subclasses analysis were recorded. Only polyclonal increases of IgG4 were considered. Summary of the Results. On 646 IgG subclass analysis performed, 59 patients had serum IgG4 over 1.35 g/L. The final diagnosis associated to serum IgG4 increase was very variable. Most patients (25%) presented with repeated infections, 13.5% with autoimmune diseases, and 10% with IgG4-RD. Other patients presented with cancer, primary immune deficiencies, idiopathic interstitial lung disease, cystic fibrosis, histiocytosis, or systemic vasculitis and 13.5% presented with various pathologies or no diagnosis. Mean IgG4 levels and IgG4/IgG ratio were higher in IgG4-RD than in other pathologies associated to elevated IgG4 levels. Conclusions. Our study confirms that elevation of serum IgG4 is not specific to IgG4-RD. Before retaining IgG4-RD diagnosis in cases of serum IgG4 above 1.35 g/L, several other pathological conditions should be excluded.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Serum IgG4 levels in different final diagnostic categories of patients with serum IgG4 elevation. IgG4-RD = IgG4-related disease. Horizontal bars represent median values observed in each group. Results obtained in each group were compared to results obtained in IgG4-RD group (Mann-Whitney test): **P < 0.005; *P < 0.05; ns: not significant.
Figure 2
Figure 2
Serum IgG4/serum IgG ratios in different final diagnostic categories of patients with serum IgG4 elevation. IgG4-RD = IgG4-related disease. Horizontal bars represent median values observed in each group. Results obtained in each group were compared to results obtained in IgG4-RD group (Mann-Whitney test): *P < 0.05; ns: not significant.
See this image and copyright information in PMC

References

    1. Hamano H, Kawa S, Horiuchi A, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. New England Journal of Medicine. 2001;344(10):732–738. - PubMed
    1. Aalberse RC, Van Milligen F, Tan KY, Stapel SO. Allergen-specific IgG4 in atopic disease. Allergy. 1993;48(8):559–569. - PubMed
    1. Hussain R, Poindexter RW, Ottesen EA. Control of allergic reactivity in human filariasis: predominant localization of blocking antibody to the IgG4 subclass. Journal of Immunology. 1992;148(9):2731–2737. - PubMed
    1. Shirakata Y, Shiraishi S, Sayama K, Miki Y. Subclass characteristics of IgG autoantibodies in bullous pemphigoid and pemphigus. Journal of Dermatology. 1990;17(11):661–666. - PubMed
    1. Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas—an autonomous pancreatic disease? The American Journal of Digestive Diseases. 1961;6(7):688–698. - PubMed

LinkOut - more resources