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Allergy. 2014 Feb;69(2):269-272. doi: 10.1111/all.12320. Epub 2013 Nov 25.

Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease.

Author information

Center for Cancer Research, Massachusetts General Hospital, Boston MA.
Università Vita-Salute San Raffaele, School of Medicine, Unit of Medicine and Clinical immunology, Milan, Italy.
Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston MA.
Contributed equally


IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that can affect virtually every organ system. T-helper type 2 responses have been presumed to be pathogenic in this disease, and a high proportion of patients with IgG4-RD are reported to have longstanding allergies, peripheral blood eosinophilia, and serum IgE elevation. It has therefore been proposed that allergic mechanisms drive IgG4-RD. However, no epidemiological assessment of atopy, peripheral blood eosinophilia, and serum IgE concentrations has ever been undertaken in patients with IgG4-RD. In this study, we evaluated these parameters in a large cohort of patients with IgG4-RD in whom a wide range of organs were affected by disease. Our results demonstrate that the majority of patients with IgG4-RD are nonatopic. Nevertheless, a subset of nonatopic subjects exhibit peripheral blood eosinophilia and elevated IgE, suggesting that processes inherent to IgG4-RD itself rather than atopy per se contribute to the eosinophilia and IgE elevation observed in the absence of atopy.


IgG4-related disease; Th2 response; allergy; atopy; eosinophilia

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