[Chronic bilateral orbital tumors in IgG4-related disease].
BACKGROUND
The IgG4-related disease describes a new multisystem disorder, which can also be manifested in the orbit. This syndrome is characterized by a multifocal chronic fibroinflammation with high levels of plasma cells expressing IgG4.


METHODS
This article reports the case of a patient with advanced bilateral tumors of the upper eyelid and orbit. The diagnosis of IgG4-related disease was only revealed after many years and after many biopsies of the tumors had been carried out. The initiation of a systemic steroid therapy led to a rapid reduction of the tumors and an improvement in the general condition of the patient.
Universitätsaugenklinik, Goethe-Universität, Frankfurt am Main, Deutschland.|Augenärztliche Gemeinschaftspraxis Prof. Reichel/Hr.Longère, Konstanz, Deutschland.
Maike
M
Rohr
Universitätsaugenklinik, Goethe-Universität, Frankfurt am Main, Deutschland.
Thomas
T
Kohnen
Universitätsaugenklinik, Goethe-Universität, Frankfurt am Main, Deutschland. Burkhard.vonjagow@schlosspark-klinik.de.|Abteilung für Augenheilkunde, Schlosspark-Klinik Berlin, Heubnerweg 2, 14059, Berlin, Deutschland. Burkhard.vonjagow@schlosspark-klinik.de.
Burkhard
B
von Jagow
D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology*; D002908:Chronic Disease; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D009918:Orbital Neoplasms / Q000175:diagnosis* / Q000276:immunology*; D013577:Syndrome
D007074:Immunoglobulin G
Fibroinflammatory disease; Lacrimal glands; Multisystem disease; Plasma cells; Systemic steroids