Promotie S.E. (Sanne) Detiger

Brief summary: 

Orbital inflammation can arise from various conditions, making diagnosis tricky due to similar symptoms. This thesis dives into understanding and treating three specific types: IgG4-related orbital disease (IgG4-ROD), orbital xanthogranulomatous disease (AOXGD), and Graves' orbitopathy (GO). In the study's first part, the challenges of diagnosing orbital inflammation are discussed. Symptoms often overlap, requiring additional tests for accurate diagnosis. The clinical features are detailed and different treatment options for each condition are suggested. Moving on, the second part explores the tissue's microscopic changes in affected patients. By using specific staining techniques, additional clues were identified helpful in diagnosing IgG4-ROD. Indications for genetic mutations were found in all subtypes of AOXGD, shedding light on potential causes. Part three delves into the body's responses during orbital inflammation and how different treatments affect outcomes. Promisingly, a steroid-sparing drug called rituximab showed effectiveness against IgG4-ROD. In the treatment of AOXGD surgical removal was sufficient for one subtype, but others required systemic therapy. A promising new drug for the treatment of GO showed reduced inflammatory symptoms with fewer side effects and a lighter treatment burden. The fourth part highlights unusual presentations of IgG4-related disease. Despite intensive treatment, some patients faced irreversible vision loss. Lastly, the thesis summarizes its main findings, emphasizing the importance of recognizing and treating these conditions early. It suggests new diagnostic tools and innovative treatments to improve patient care in the future.