IgG4-related disease and systemic vasculitis – is there any connection?

• Main Authors: Anna Masiak, Zbigniew Zdrojewski
• Format: Article
• Language: English
• Published: Termedia Publishing House 2014-11-01
• Series: Rheumatology
• Subjects: IgG4-related diseases; systemic vasculitis; ANCA antibody
• Online Access: http://www.termedia.pl/IgG4-related-disease-and-systemic-vasculitis-is-there-any-connection-,18,24059,1,1.html

IgG4-related disease is a relatively new group of diseases of still unknown etiology. It is characterized by elevated serum levels of subclass IgG4 immunoglobulin and by abundant infiltration of IgG4+ plasma cells with typical fibrosis of the affected organs. Elevated concentration of IgG4 may be present in many other conditions associated with chronic inflammation. In recent years, it is noted that this may also apply to patients with systemic vasculitis, in particular antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The aim of this study is to draw attention to the fact that in some cases, both clinical presentation and histopathological findings in IgG4-related diseases and systemic vasculitis may be similar. The importance of elevated serum IgG4 immunoglobulin in patients with ANCA-associated vasculitis (AAV) is unclear and requires further research.