IgG4-Related Disease

Immunoglobulin G4-related disease (IgG4-RD) is…

IgG4-Related Disease: a recently described disease, but its known clinical manifestations are from of old. Finally, after all these decades, a single pathology links disparate conditions like Sicca Syndrome (non-Sjogren’s), retroperitoneal fibrosis, sclerosing cholangitis, autoimmune pancreatitis, Riedl’s thyroiditis, aortitis, interstitial pneumonia, tubulointerstitial nephritis, etc.
Interestingly, while IgG4-producing plasma cells are pathonomonic of this disease, IgG4 itself is not pathogenic.

IgG4-related disease and its pathogenesis—cross-talk between innate and acquired immunity

IgG4-related disease (IgG4-RD) is a novel clinical entity proposed in Japan in the 21th century and is attracting strong attention over the world. The characteristic manifestations of IgG4-RD are increased serum IgG4 concentration and tumefaction by IgG4+ plasma cells. Although the clinical manifest…