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Diffuse bilateral orbital inflammation in Churg- Strauss syndrome
Thanuja Gopal Pradeep1, Venkatesh C Prabhakaran, Alan McNab
1South Australian Institute of Ophthalmology and Discipline of Ophthalmology and Visual Sciences, Royal Adelaide Hospital, University of Adelaide, Adelaide, South Australia. thanugopal@yahoo.co.in
Ophthalmic Plastic and Reconstructive Surgery
|January 22, 2010
Summary
Churg-Strauss syndrome can manifest as orbital inflammation in asthma patients. Early diagnosis and biopsy are crucial for effective treatment and preventing complications.
Area of Science:
- Ophthalmology
- Rheumatology
- Pathology
Background:
- Churg-Strauss syndrome (CSS), also known as eosinophilic granulomatosis with polyangiitis (EGPA), is a rare systemic vasculitis.
- It is characterized by asthma, eosinophilia, and granulomatous inflammation.
Observation:
- Two cases of CSS presenting with diffuse bilateral orbital inflammation are described.
- Patients exhibited symptoms such as periocular swelling, proptosis, and conjunctival nodules.
- Orbital imaging revealed enlarged extraocular muscles, lacrimal glands, and orbital fat infiltration.
Findings:
- Blood tests showed positive p-antineutrophil cytoplasmic autoantibodies and significant eosinophilia.
- Orbital biopsies confirmed granulomatous inflammation with eosinophil predominance and vasculitic changes.
- Both patients responded well to corticosteroid therapy, with resolution of systemic and ocular findings.
Implications:
- Orbital inflammation in asthma patients with eosinophilia suggests CSS.
- Prompt diagnosis through biopsy is essential for timely intervention.
- Early treatment can mitigate severe systemic and ophthalmic complications associated with CSS.
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