Orbital inflammatory pseudotumor revealing multi‐organ immunoglobulin G4‐related disease

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an immunemediated fibro-inflammatory condition that is characterized by extensive storiform fibrosis, obliterative phlebitis, thick lymphoplasmacytic infiltrates with a high percentage of IgG4-bearing plasma cells, and frequent tissue eosinophilia. 1 This disorder, which can affect practically any organ synchronously or metachronously, can result in the development of sclerotic masses of varying sizes.Differentiating IgG4-RD from other rheumatic diseases is challenging for clinicians. Currently, IgG4-RD is recognized as an important cause of idiopathic orbital inflammation (IOI) which must be differentiated from other pathologies that include Hodgkin's lymphoma and non-Hodgkin's lymphoma, granulomatosis with polyangiitis (Wegener's), Graves' orbitopathy, and other conditions. [1][2][3][4] Their incidence was estimated at 4.7%-6.3% of all orbital diseases. 5 We report a case of multi-organ IgG4-RD revealed by an orbital IOI.

IgG4‐related disease presenting as severely symptomatic hypercalcemia: A case report and review of literature

“…Immunoglobulin G4‐related disease (IgG4‐RD) is an insidiously progressive autoimmune fibro‐inflammatory condition characterized by tumorfactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4‐positive plasma cells, storiform fibrosis, and, often but not always elevated serum IgG4 concentrations 1 . It can affect the pancreas, salivary glands, orbit, and lymph nodes, 2 and idiopathic retroperitoneal fibrosis is common with IgG4‐RD 3 . IgG4‐RD is highly treatable and responds promptly to glucocorticoids.…”