IgG4-Related Disease

Abstract

IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by an elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4-RD encompasses a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), hypophysitis, Riedel thyroiditis, tubulointerstitial nephritis, prostatitis, retroperitoneal fibrosis, inflammatory aortic aneurysm and inflammatory pseudotumor. However, like a crow flying on a dark night, IgG4-RD passed through the history of medicine, until the concept of an overarching of these systemic disorders was established in Japan at the beginning of the present century. In addition, most clinical practitioners are not yet familiar with IgG4-RD, and thus one cannot diagnose what one does not know. The purpose of the book [1] is to raise awareness of this disease and its diagnostic pitfalls. Another goal was to delineate the characteristic features of individual organs in IgG4-RD radiologically and histopathologically with many color photographs. Most of the authors are members of the IgG4 team of the Japanese the Ministry of Health, Labor and Welfare (MHLW), and experts who have published numerous important papers in the field. Thus, this book will be useful to physicians in various disciplines such as gastroenterology, rheumatology, ophthalmology, otolaryngology, urology, hematology, respiratory medicine and oral medicine, not only as a textbook but also as an authoritative and comprehensive reference work.