Abstract
IgG4-Related Disease (IgG4-RD) is a multiorgan, immune-mediated, fibro-inflammatory disease of unknown etiology that has integrated a spectrum of conditions previously not recognized as related to each other or any systemic disease. It has an estimated prevalence of 4.6 per 100,000, however, the exact prevalence of IgG4-RD is difficult to estimate since there have been few published studies, and these studies used different criteria for diagnosis, tended to enroll small groups of patients with heterogeneous pathology, and had uncertain generalizability [1,2].
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