IgG4-gerelateerde ziekte

Conflict of interest: disclosure forms provided by the authors are available along with the full text of this article at www.ntvg.nl, search for A6476; click on ‘Belangenverstrengeling’ (‘Conflict of interest’). Financial support for this article: L.J. Maillette de Buy Wenniger has received an AMC PhD Scholarship from the Academic Medical Center-University of Amsterdam and a Gastrostart grant from the Dutch Society for Gastroenterology (Nederlandse Vereniging voor Gastro-enterologie) for the research described in this article.

• The diagnosis IgG4-related disease (IgG4-RD) is often difficult to make. The clinical spectrum is diverse, with a variety of organ systems that may be affected simultaneously or sequentially.

• Patients often present with symptoms that mimic a malignant disease, for example, symptoms compatible with a pancreatic tumour.

• The lack of reliable tests often prolongs the diagnostic process.

• Limited insight into the causative disease mechanisms has confined the therapeutic options to the empirical use of immunosuppression.

• During the past year, the first papers on the fundamental aspects of the disease have resulted in the emergence of a new disease model for IgG4-RD.

• Recently published clinical and experimental findings support the hypothesis that antigenic stimulation plays a pivotal role in the aetiology of IgG4-RD.

• These new insights may pave the way for more sensitive diagnostic tests and more evidence-based strategies to cope with the many manifestations of IgG4-related disease.