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IgG4-assoziierte orbitale Entzündungen


Schweier, C; Kordic, H; Chaloupka, K (2013). IgG4-assoziierte orbitale Entzündungen. Klinische Monatsblätter für Augenheilkunde, 230(4):370-373.

Abstract

INTRODUCTION:
Serum immunoglobulin G4 (IgG4)-related systemic disease is a newly found entity and should be considered as a further differential diagnosis to the big pool of unspecific orbital inflammation. We describe clinical appearance and treatment options.
PATIENTS AND METHODS:
We retrospectively analyzed all patients with the diagnosis IgG4-related orbital inflammation who were examined in our outpatient clinic. We compared symptoms, clinical and histological findings and therapy.
RESULTS:
In the last 17 months five patients were diagnosed with IgG4-related orbital disease. Biopsies were performed of most affected areas as seen in the computerised tomography scan. Histology revealed marked infiltration with IgG4-positive plasma cells. Serum IgG4-levels were partly significantly elevated. Based on these findings diagnosis of an IgG4-related disease was agreed and treatment with oral prednisone installed. In one patient a therapy with Rituximab was successfully started.
CONCLUSION:
Extended unspecific orbital inflammation, especially when bilateral and associated with systemic disease should be considered to be IgG4-related. Histological proof of IgG4-positive plasma cells and elevated IgG4 serum levels do confirm this diagnosis. Strict follow-up and interdisciplinary work-up is recommended.

INTRODUCTION:
Serum immunoglobulin G4 (IgG4)-related systemic disease is a newly found entity and should be considered as a further differential diagnosis to the big pool of unspecific orbital inflammation. We describe clinical appearance and treatment options.
PATIENTS AND METHODS:
We retrospectively analyzed all patients with the diagnosis IgG4-related orbital inflammation who were examined in our outpatient clinic. We compared symptoms, clinical and histological findings and therapy.
RESULTS:
In the last 17 months five patients were diagnosed with IgG4-related orbital disease. Biopsies were performed of most affected areas as seen in the computerised tomography scan. Histology revealed marked infiltration with IgG4-positive plasma cells. Serum IgG4-levels were partly significantly elevated. Based on these findings diagnosis of an IgG4-related disease was agreed and treatment with oral prednisone installed. In one patient a therapy with Rituximab was successfully started.
CONCLUSION:
Extended unspecific orbital inflammation, especially when bilateral and associated with systemic disease should be considered to be IgG4-related. Histological proof of IgG4-positive plasma cells and elevated IgG4 serum levels do confirm this diagnosis. Strict follow-up and interdisciplinary work-up is recommended.

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1 citation in Web of Science®
1 citation in Scopus®
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Additional indexing

Other titles:IgG4-related orbital inflammation
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Ophthalmology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:German
Date:2013
Deposited On:25 Oct 2013 06:35
Last Modified:05 Apr 2016 17:04
Publisher:Georg Thieme Verlag
ISSN:0023-2165
Publisher DOI:https://doi.org/10.1055/s-0032-1328360
PubMed ID:23629783
Permanent URL: https://doi.org/10.5167/uzh-82226

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