INVESTIGATION OF IGG4-RELATED DISEASE PREVALENCE IN PATIENTS WITH IDENTIFIED DIAGNOSIS OF IDIOPATHİC ORBITAL INFLAMMATION


Thesis Type: Expertise In Medicine

Institution Of The Thesis: Gazi University, Tıp Fakültesi, GÖZ HASTALIKLARI ANABİLİM DALI, Turkey

Approval Date: 2018

Thesis Language: Turkish

Student: GÖKÇEN DENİZ GÜLPINAR İKİZ

Supervisor: Onur Konuk

Abstract:

The purpose of this study is; retrospective analysis of patients diagnosed

with orbital myositis, dacryioadenitis, scleritis, Tolosa-Hunt syndrome or

pseudotumor orbita, under the heading of idiopathic orbital inflammation (IOI) and

to determine the prevalence of IgG4-related orbital disease in this group of patient.

Between the years 2003 and 2017, 133 patients enrolled with IOI and

reactive lymphoid hyperplasia in the Ophthalmology and Orbital Surgery

Department of Gazi University Faculty of Medicine (GÜTF) were reviewed

retrospectively. 3 patients diagnosed with lenfoma and exculuded. Serum samples

were taken from the patients who came for control visits and evaluated with the

IgG4 ELISA kit in the GÜTF Biochemistry Laboratory. Histopathological

diagnosis of orbital biopsy specimens were recorded. Medical files of 130

patients(47 male, 83 female) who were diagnosed with idiopathic orbital

inflammation, were retrospectively reviewed. The ages of the cases were in the

range of 4-80 years and the average age was 40.9 ± 1,4. It has been shown that after

2010, the prevalence of the disease increases, autoimmune thyroid disease can be

accompanied, and they are frequently admitted to the clinic with acute pain. The

cases are confronted with diplopia, especially eyelid edema, conjunctiva

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hyperemia, proptosis, limitation of eye movement, and decrease in visibility at least

occasionally. For the localized disease, extraocular muscle involvement was

remarkably the most common finding, where, medial rectus is the most common

site of inflammation. Lacrimal gland involvement was seen at the second most

frequent occurrence. It was determined that the main treatment regimen was

systemic steroid administration, with this treatment, the success rate was found to

be %95, in which recuurence rate was 13.1%. 18 of the 33 patients who had tissue

specimens optimally analysed with IgG4 tissue dye, were histopathologically IgG4

positive and IgG4 serum marker positivity was found to be 66.7% in this group.

Retrospective analysis of the clinical characteristics of IOI patients

revealed that the data are consistent with the disease profile in the literature. On the

other hand, when compared with the total number of IOI patients, the number of

patients having histopathologic samples and serum marker levels are limited, which

is an important scantiness when evaluating the prevalence of IgG4-related disease

as a subgroup. Even within this limited sample group, 54.5% of the IgG4-related

disease rate supports the fact that a significant proportion of the IOI patient group

may actually be the orbital manifestation of the IgG4-related disease, as noted in

the literature. For this reason, it is necessary to conduct studies to search for

noninvasive tests that are more sensitive and more reliable than serum marker levels

in the diagnosis of IgG4 related disease.