Granulomatosis with Polyangiitis with Ocular Manifestations

Abstract

Granulomatosis with polyangiitis (GPA) is a granulomatous-necrotic systemic vasculitis with a lesion of predominantly the upper and lower respiratory tracts at the onset of the disease (vasculitis, accompanied by granulomatous inflammation), and subsequently renal (glomerulonephritis). In addition, GPA may manifest as inflammation of small arteries and veins. Despite many years of study of this disease, the etiology of GPA remains unknown. The present case is about a 47-year-old female, who had been suffering from necrotizing scleritis, corneal ulcer, and secondary glaucoma in both eyes for 3 months, and she was treated with anti-inflammatory and antimicrobial therapy that showed no effect; the patient's general condition became worse. In the second week of treatment, multiple abscess ruptures exposed the sclera. Sampling of the affected conjunctival tissue and positive HLA B8 haplotype and ANCA (PR3-ANCA) testings make it clear that GPA was the main reason of necrotizing scleritis with inflammation. The targeted treatment of the underlying disease allows to stabilize an inflammation of corneal and scleral lesions.

Keywords: Corneal ulcer; Granulomatosis with polyangiitis; Necrotizing scleritis; Wegener's granulomatosis.

Fig. 1

The condition of the right and left eyes at admission: subconjunctival hemorrhage, chemosis, dense, in the form of a “roller” infiltration of the conjunctiva, partially covering the limb zone (a, b); zone of corneal ulceration (white arrow) (c, d).

Fig. 2

Right eye. Keratotopography (OCULUS Pentacam®) showed a deep defect of the corneal stroma in the paralimbal zone, on the contrary, in the adjacent corneal paraoptic zone, there was a sharp elevation of the lunate shape due to perifocal edema and cicatricial process.

Fig. 3

Right eye. Optical coherence tomography (Heidelberg Spectralis®) a zone of sharp thinning of the stroma in the paralimbal zone, the structure of the stroma was loose, adjacent to the zone of the ulcer defect was accompanied by a zone of perifocal edema. Similar changes were observed at all levels of scanning along the limbal zone.

Fig. 4

Left eye. In the paralimbal zone with the established zone of destruction of the deep layers of the stroma, covered with a tear film. Peripheral damage was noted as “stepped,” which was biomicroscopically corresponding to the active edge of an ulcerative defect. In contrast to the heterogeneous structure of the endothelium, corresponding to single precipitates.

Fig. 5

MRI demonstrated right-sided sinusitis, frontal sinusitis, and residual signs of sphenoiditis and edema of eye muscles on the left eye.

Fig. 6

Abscessing and spontaneous opening of the abscess with the presence of mucopurulent discharge.

Fig. 7

Hematoxylin and eosin stain. 20× of biopsy showing a patchy infiltrative pattern. Chronic granulomatous inflammatory infiltrate and focal areas of necrosis (white arrows).