Manifestation of IgG4 Associated Disease in the Head and Neck Region


Authors: J. Hybášková 1;  K. Zeleník 1,2;  O. Urban 2,3;  P. Vítek 2,4;  P. Matoušek 1,2;  P. Komínek 1,2
Authors‘ workplace: Klinika otorinolaryngologie a chirurgie hlavy a krku, Fakultní nemocnice Ostrava, přednosta prof. MUDr. P. Komínek, Ph. D., MBA 1;  Lékařská fakulta, Ostravská univerzita, vedoucí katedry prof. MUDr. P. Komínek, Ph. D., MBA 2;  Centrum péče o zažívací trakt, Vítkovická nemocnice, Ostrava, primář MUDr. O. Urban, Ph. D. 3;  Interní oddělení, Nemocnice ve Frýdku-Místku, primář MUDr. P. Vítek, Ph. D. 4
Published in: Otorinolaryng. a Foniat. /Prague/, 64, 2015, No. 3, pp. 163-167.
Category: Review Article

Overview

Immunoglobulin G4 – related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder. Although patients have been described more then 100 years ago, the systemic nature of this disease has been recognized in the 21st century only. IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum and many others. Recent research enabled a clear clinical and histopathological description of IgG4-RD. Typically, lymphoplasmacellular inflammation, storiform fibrosis and obliterative flebitis, are found in IgG4-RD biopsies. Elevated serum IgG4 levels are found in many but not all patiens. Treatment is based on clinical experience. Glucocorticoids are the mainstay of therapy, although adjunctive immunosuppressive agents are used in relapsing patient.

Keywords:
IgG4-RD, immunoglobulin 4, storiform fibrosis, lymphoplasmacytis inflammations


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Audiology Paediatric ENT ENT (Otorhinolaryngology)
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