BACKGROUND: The IgG4-related systemic disease as well as the homonymous variant IgG4-related orbital disease were first described less than 15 years ago. The mostly subacute clinical symptoms can be multifarious and the classical case is characterized by an orbital inflammatory condition with a bilateral enlargement of the lacrimal glands; however, any other orbital tissue with the exception of the eyeball can be affected by the lymphocytic inflammatory infiltration. MATERIAL AND METHODS: Based on the current literature the clinical picture, epidemiology, pathogenesis and treatment options are described. A focus is on the differential diagnostic demarcation from other inflammatory processes of the orbit. CONCLUSION: The IgG4-related orbital disease is an important differential diagnosis of inflammatory diseases of the orbit. The condition can exhibit considerable clinical and imaging similarity to idiopathic inflammation of the orbit, to the specific inflammations seen in systemic diseases, to Graves' orbitopathy and to lymphoproliferative diseases and lymphoma. After histopathologic confirmation the interdisciplinary clarification and treatment consensus are indispensable.
BACKGROUND: The IgG4-related systemic disease as well as the homonymous variant IgG4-related orbital disease were first described less than 15 years ago. The mostly subacute clinical symptoms can be multifarious and the classical case is characterized by an orbital inflammatory condition with a bilateral enlargement of the lacrimal glands; however, any other orbital tissue with the exception of the eyeball can be affected by the lymphocytic inflammatory infiltration. MATERIAL AND METHODS: Based on the current literature the clinical picture, epidemiology, pathogenesis and treatment options are described. A focus is on the differential diagnostic demarcation from other inflammatory processes of the orbit. CONCLUSION: The IgG4-related orbital disease is an important differential diagnosis of inflammatory diseases of the orbit. The condition can exhibit considerable clinical and imaging similarity to idiopathic inflammation of the orbit, to the specific inflammations seen in systemic diseases, to Graves' orbitopathy and to lymphoproliferative diseases and lymphoma. After histopathologic confirmation the interdisciplinary clarification and treatment consensus are indispensable.
Authors: Heounjeong Go; Ji Eun Kim; Young A Kim; Ho Kyung Chung; Sang In Khwarg; Chul-Woo Kim; Yoon Kyung Jeon Journal: Histopathology Date: 2012-01 Impact factor: 5.087
Authors: Luca Iaccarino; Rosaria Talarico; Carlo Alberto Scirè; Zahir Amoura; Gerd Burmester; Andrea Doria; Karim Faiz; Charissa Frank; Eric Hachulla; Miguel Hie; David Launay; Carlomaurizio Montecucco; Sara Monti; Luc Mouthon; Angela Tincani; Paola Toniati; Pieter Martin Van Hagen; Ronald F Van Vollenhoven; Stefano Bombardieri; Ulf Mueller-Ladner; Matthias Schneider; Vanessa Smith; Maurizio Cutolo; Marta Mosca; Tobias Alexander Journal: RMD Open Date: 2019-01-19
Authors: Julia Walkowska; Nicol Zielinska; Piotr Karauda; R Shane Tubbs; Konrad Kurtys; Łukasz Olewnik Journal: J Clin Med Date: 2022-09-22 Impact factor: 4.964