Granulomatosis with Polyangiitis Presenting with Bilateral Orbital Apex Syndrome

Abstract

We report a rare case of granulomatosis with polyangiitis (GPA) presenting with bilateral orbital apex syndrome (OAS). A 73-year-old woman with a history of endoscopic sinus surgery for ethmoidal sinusitis experienced a sudden decrease in visual acuity (VA) of both eyes. At the initial examination, her VA had decreased to 0.01 in the right eye and 0.03 in the left eye, and eye movement in both eyes was mildly limited in all directions. Visual field tests of both eyes showed a large central scotoma. Laboratory tests revealed an elevation of myeloperoxidase-anti-neutrophil cytoplasmic antibody. Facial computed tomography demonstrated a thickened mucosal membrane in the entire ethmoidal sinus, and the posterosuperior walls of Onodi cells filled with infiltrative lesions had thinned. Orbital magnetic resonance imaging showed severe inflammation in the orbital apex. From these clinical findings, the patient was diagnosed with GPA presenting with OAS associated with ethmoid sinusitis. Emergent endoscopic sinus surgery was performed for biopsy and debridement of the ethmoidal and sphenoid sinusitis to decompress the optic nerve. One day after endoscopic sinus surgery, the patient’s VA and visual field were improved, and steroid pulse therapy was commenced postoperatively. Four days later, VA had recovered to 1.0 in both eyes, and eye movement and visual field had were improved. Although OAS is a rare manifestation, early surgical treatment should be considered when the orbital lesion presents as risk of rapid deterioration of visual function in patients with GPA.

A Case of an Elderly Woman Who Developed Corneal Perforation in the Clinical Course of Myeloperoxidase Positive Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

“…However, in our patient, although C-reactive protein and MPO-ANCA titers at the time of previous relapse of scleritis were obviously increased, they did not increase at the time of recent scleritis relapse with corneal perforation. We searched for previous cases of patients with AAV with ocular lesions and extracted case reports that described C-reactive protein and ANCA titers [12,[16][17][18][19][20][21][22][23][24][25][26][27]. We then investigated the relationship between patients with AAV with ocular lesions and Creactive protein levels, as well as ANCA titers (Table 2).…”

Autoimmune Diseases and Retina

“…Özellikle, ANCA için bir kan testi, orbital ve pulmoner inflamatuar lezyonu saptamak için BT veya MRI ile bir görüntüleme testi ve histolojik inceleme, GPA'nın kesin tanısını koymak için kritik öneme sahiptir. [80]…”

Bilateral Orbital Apex Syndrome Due to Granulomatosis with Polyangiitis

“…2 Of ocular presentations, orbital involvement is most frequent at 22%-45%. 3 Orbital manifestations may be secondary to primary granulomatous vasculitis or contiguous sinus involvement. 2 The latter mechanism is more common; infiltration from paranasal sinuses is demonstrated in 72% of orbital GPA cases.…”

“…In most of these cases, OAS was caused by contiguous sinus or pachymeningeal infiltration. [3][4][5][6][7][8] OAS without contiguous sinus spread has been reported, but occurred in a patient with a pre-existing confirmed diagnosis of GPA. 9 This case highlights several key points.…”