Abstract

Aim:a The spectrum of histopathological changes of immunoglobulin G4-related orbital diseases (IgG4-RDs), summarise the clinical manifestations & imaging features of the IgG4-RODs & explore the early diagnosis and management of IgG4RD.
Methods: 15 Orbitotomy specimens which were diagnosed as IgG4RD based on HPE & IHC were included in the study, correlation of demographics, site of involvement,laterality,serum IgG4 levels,systemic involvement response to steroid &immunomodulators were correlated
Results:Mean age group of IgG4RD was 42 years youngest patient was 14 year old, lacrimal gland was the most common orbital structure involved,1 case had systemic involvement,12 cases had classical HPE features while only 9 out of the 15 cases had associated raised serum IgG4 levels
Conclusion:Orbital IgG4 is frequently misdiagnosed and wrongly labelled orbital pathology,deep understanding of its pathogenesis & clinicopathological features