IgG4-gerelateerde ziekte

• 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.