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Topic: ESOPRS 2021 ePoster sessions
Time: Sep 17, 2021 16:00 Amsterdam, Berlin, Rome, Stockholm, Vienna, 15:00 London

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A complex case of orbital presentation of Granulomatosis with polyangiitis

Author: Victoria Ariasgago
ePoster Number: 288

Purpose

To describe a case of Granulomatosis with polyangiitis(GPA) with orbital presentation

Methods

Descriptive case report and literature review

Results

A 44-year-old male with a history of episcleritis,lacrimal glands enlargement,otitis media, and facial nerve palsy,consulted with proptosis,painful red eye and respiratory distress. Visual acuity was 20/20 in both eyes, with decreased ocular motility, diplopia,eyelid edema and episcleritis.Intraocular pressure was 20mmHg in the right eye and 14mmHg in the left eye. The fundoscopy was normal. Proptosis was 23mm in the right eye and 21mm in the left eye. Laboratory tests revealed elevation of C- ANCA in blood and pulmonary chest images showed pulmonary involvement.He was diagnosed as GPA and pulses of methylprednisolone and cyclophosphamide were initiated.Rituximab was indicated for maintenance.Two weeks later, his vision decreased to 20/40 in the right eye and no light perception in the left eye, afferent pupillary defect, and increased proptosis with limitation of ocular motility in all gaze positions. New CT scan showed total infiltration of both orbits. Pulse therapy with methylprednisolone one gram per day for 5 days was indicated, with no improvement,so a left three-wall orbital decompression was performed with biopsy.The pathology shows fatty tissue necrosis with small granulomas of epithelioid histiocytes and extensive areas of fibrosis. Vasculitis foci of small vessels and mixed inflammatory infiltrate.

Conclusion

GPA is the multisystemic vasculitis most frequently associated with orbital involvement.It affect young adults without sex distinction.The main features are elevated serum ANCAs and gigant cells granulomas with necrosis, and vasculitis.It can affect all eye structures, but the orbit is the most affected.On CT scan orbital granulomas can be found, with bone erosion.Septal deviation with bone destruction of the the nose and masses in the orbit are suggestive of GPA.Treatment consists of corticosteroids with cyclophosphamide to induce remission, and maintenance therapy with rituximab, methotrexate, or azathioprine.

Additional Authors

Abstract ID: 21-195