Abstract ID: 26-346
Adult-onset Asthma and Periocular Xanthogranulomas Syndrome Associated with IgG4-related Disease and Response to Rituximab Treatment: A Case Report and Literature Review
Author: Rand Alazaz Base Hospital / Institution: King Khaled Eye Specialist Hospital
Presentation Type: ePoster Presentation
Purpose
Introduction: Adult orbital xanthogranulomatous disease (AXOGD) includes AGX, AAPOX, NXG, and ECD, involving non-Langerhans histiocytic proliferation with foamy histiocytes and Touton giant cells. AAPOX, linked to IgG4-RD, presents as periocular swelling, often with systemic conditions like asthma or lymphomas. Diagnosis involves biopsy and serum IgG4 testing. Corticosteroids are first-line treatment, but steroid-sparing agents like rituximab are effective, especially in refractory cases or those with steroid-related complications. Previous literature identifies Rituximab as effective for treating IgG4-related disease, particularly adult-onset asthma with periocular xanthogranulomas, by reducing inflammation, fibrosis, and disease activity, offering a comprehensive therapeutic approach for refractory cases.
Methods
NA
Results
Case Detail: A 56-year-old female presented with bilateral lacrimal gland enlargement and recurrent swelling worsening upon steroid tapering, leading to complications like weight gain and Cushing syndrome. She has a history of hypothyroidism, asthma, and obesity. Clinical findings included limited eye movement and asymmetrical cup-to-disc ratio. MRI showed marked lacrimal gland enlargement, extraocular muscle involvement, and fat infiltration. Histopathology revealed lymphoid and plasma cell infiltration, Touton giant cells, and collagenous fibrosis, with positive IgG/IgG4 staining. Negative tests for ANA, ANCA, and ACE ruled out other conditions. The patient received a combination of oral prednisolone and rituximab which resulted in clinical remission.
Conclusion
Conclusion: This case highlights the diagnostic complexity of AAPOX linked to IgG4-RD, emphasizing the importance of histopathology and imaging. Rituximab emerges as an effective treatment for refractory cases, reducing inflammation and disease activity, underscoring the need for personalized, integrated management strategies to prevent complications and irreversible organ damage.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Nada | Almadhi | King Khaled Eye Specialist Hospital |