Zusammenfassung
Hintergrund
Sowohl die Ig(Immunglobulin)G4-assoziierte Erkrankung der Orbita als auch die gleichnamige Systemerkrankung sind erst seit weniger als 15 Jahren beschrieben. Die meist subakute Klinik kann vielgestaltig sein und ist im klassischen Fall durch eine entzündliche orbitale Symptomatik mit bilateraler Vergrößerung der Tränendrüsen gekennzeichnet. Es können aber auch alle anderen Gewebe der Orbita mit Ausnahme des Bulbus von den lymphozytären entzündlichen Infiltraten betroffen sein.
Material und Methoden
Anhand aktueller Literatur werden Klinik, Epidemiologie, Pathogenese und Therapie dieses Krankheitsbildes erläutert. Ein Schwerpunkt liegt dabei auch auf den differenzialdiagnostischen Abgrenzungen zu anderen Orbitaprozessen.
Diskussion
Die IgG4-assoziierte Erkrankung stellt eine wichtige Differenzialdiagnose der entzündlichen Erkrankungen der Orbita dar und kann klinisch und bildgebend große Ähnlichkeit zur idiopathischen orbitalen Entzündung, den spezifischen Entzündungen im Rahmen von Systemerkrankungen, der endokrinen Orbitopathie, aber auch lymphoproliferativen Erkrankungen/Lymphomen aufweisen. Nach histologischer Sicherung sind interdisziplinäre Abklärung und Therapieabstimmung unerlässlich.
Abstract
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.
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Pitz, S. IgG4-assoziierte Erkrankung. Ophthalmologe 118, 787–793 (2021). https://doi.org/10.1007/s00347-021-01448-8
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DOI: https://doi.org/10.1007/s00347-021-01448-8
Schlüsselwörter
- Idiopathische orbitale Entzündung
- Spezifische orbitale Entzündung
- Lymphoide Hyperplasie
- Orbitale Lymphome
- Histologische Sicherung