Immunoglobulin G4-related systemic disease (IgG4-RD) is an increasingly recognized syndrome; many clinical conditions can be attributed to this new entity. The cause is not known; age of onset is usually middle age.
As far as the clinical features are concerned the spectrum includes, similar to - Type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis, Mikulicz disease (or Mikulicz syndrome) and sclerosing sialadenitis, inflammatory orbital pseudotumor, and chronic sclerosing dacryoadenitis, Idiopathic retroperitoneal fibrosis and related disorders, Chronic sclerosing aortitis, Riedel’s thyroiditis and a subset of Hashimoto’s thyroiditis, IgG4-related interstitial pneumonitis and pulmonary inflammatory pseudotumors, IgG4-related renal disease, particularly tubulo-interstitial nephritis (TIN). Pathology: Hallmarks of IgG4-RD are lympho-plasmacytic tissue infiltration…
