Abstract
IgG4-related disease (IgG4-RD) is a clinical entity characterized by elevated serum IgG4 and tumor-like inflammation, with tissue infiltration by IgG4 and plasma cells. IgG4-RD is rare, but clinically significant, and its urologic manifestations have been reported in the literature. The present review covers a broad spectrum, describing the pathologies related to the area of urology.
 In 2003, Terumi Kamisawa was the first to recognize IgG4-RD, characterized by multiorgan lesions in patients with autoimmune pancreatitis and classified as an inflammatory and fibrotic entity with a dense lymphoplasmacytic infiltrate, positive for IgG4.(1–3) It presents in middle-aged patients, between 59-68 years of age, with no clear distribution by sex, (4–6) and has different clinical presentations. The main urologic manifestations are inflammatory pseudotumors and lower urinary tract symptoms. The present article offers a clear, general overview of the disease, encompassing its pathophysiology, diagnosis, and treatment, from the perspective of urology.
Highlights
IgG4 is an antibody with a unique structure and function and makes up less than 5% of the total IgG in healthy individuals
Homology has been described between human carbonic anhydrase and the alfa carbonic anhydrase of Helicobacter pylori and their homologous segments join to the HLA DRB1*0405, activating the immune system
A connection exists between the plasminogen protein-ligand of Helicobacter pylori and ubiquitin ligase E with the n-recognin 2 component that pancreatic acinar cells express, showing a correlation between an infectious agent and the development of autoimmune pancreatitis
Summary
IgG4 is an antibody with a unique structure and function and makes up less than 5% of the total IgG in healthy individuals. In contrast to IgG1, 2, and 3, serum IgG4 concentrations in healthy persons vary by a factor of more than 100 (reference values from 0.01 to 1.4 mg/dl) but IgG4 values do not generally fluctuate in individual persons.[1]. IgG4 binds weakly to the complement component (C1) and Fc receptors and does not play an active role in immune activation. The diagnostic criteria were proposed by the Japanese study group and validated for the worldwide population
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