A 65-year-old Caucasian male was referred to a provincial ophthalmology service with a 2-week history of bilateral eye redness, photophobia, and ocular discomfort. He also described intermittent spontaneous epistaxis, fatigue, lethargy, and unintentional weight-loss. He had a background of ischaemic heart disease, hyperlipidaemia, hypertension and right hip osteoarthritis awaiting replacement. His medication history included: aspirin, metoprolol, and atorvastatin. He was a retired truck driver and ex-smoker. He had no previous ophthalmic history of note with no prior ocular surgery.
His best corrected visual acuities on presentation were 6/5 (right) and 6/9 (left) with normal intraocular pressures. Nodular scleritis extended to the right nasal limbus (shown in Fig. 1). The cornea was clear without defect. The anterior and posterior segments were quiescent in both eyes, with normal fundus and optic nerve appearance. Systemic blood work and a punch biopsy of his right scleral mass were taken.
He subsequently represented acutely to the emergency department 10 days later with frank haematuria, dizziness, and new confusion. He displayed word-finding difficulties and atypical dysarthria leading to admission for further systemic work-up and neuroimaging.
His blood work demonstrated anti-neutrophil cytoplasmic IgG autoantibody (ANCA) positivity with an anti-proteinase 3 antibody (PR3) level of 112 U/mL. His complement studies showed C3 elevation (1.95 g/L) and normal C4 (0.46 g/L). Anti-myeloperoxidase antibody levels were <3 U/mL. Glomerular basement membrane antibodies were not detected. Infectious screen including sarcoidosis, tuberculosis, and syphilis was negative. Rheumatology factor and anti-cyclic citrullinated peptide were both negative. Coagulation studies returned normal. His creatinine was 96 mumol/L with an estimated glomerular filtration rate of 71 mL/min/1.73 m2. Urine cytology demonstrated red blood cells, polymorphonuclear leucocytes without evidence of malignant cells. Liver function tests demonstrated normal transaminase levels, however, elevated alkaline phosphatase (199 U/L) and Gamma-glutamyl transferase (164 U/L). Hepatitis B and C serology along with human immunodeficiency virus serology were negative. Multi-system computed tomography imaging demonstrated lung, liver, prostate, bladder, nasal, and ethmoidal/maxillary sinus involvement (shown in Fig. 2). Biopsies of both his right nasal sclera and nasal cavity mass demonstrated inflamed granulation tissue with reactive atypical squamous epithelium without frank dysplasia or malignancy. Multinucleate giant cells were visible in both biopsied samples. The histopathology slide of the right scleral biopsy is shown in Figure 3.
An acute infarct of the left inferior parietal cortex was present on magnetic resonance angiography without evidence of cerebral vasculitis. Stenosis of both proximal internal carotid arteries with >80% critical stenosis on the left side was revealed on duplex carotid ultrasound.
A diagnosis of granulomatous with polyangiitis was made and a multidisciplinary approach employed including ophthalmology, otolaryngology (ENT), rheumatology, urology, and vascular surgery subspecialties. He was commenced on intravenous methylprednisolone 1 g for three consecutive days. He received two, 1 g rituximab infusions 14 days apart and cotrimoxazole 960 mg 3 times-weekly. He remained on aspirin, metoprolol, and atorvastatin. Dual antiplatelet therapy was avoided due to his ongoing haematuria and epistaxis. He successfully underwent left endarterectomy.
During the initial stages of his intravenous methylprednisolone treatment, he developed right peripheral ulcerative keratitis (PUK). This occurred 12 days from presentation, 2 days after systemic steroid commencement. There was significant corneal thinning with loss of the nasal juxtalimbal corneal stroma. Due to the high risk of morbidity, corneal scraping was not conducted. His topical eye drops included g.chloramphenicol 0.5% four-times daily, g.hyaluronate sodium 0.1% every 2-hours, and g.acetylcysteine 10% four-times daily in addition to oral doxycycline and ascorbic acid. Rapidly progressive corneal thinning was noted with extensive stromal tissue loss exposing Decement's membrane. Due to the high risk of perforation, surgical temporizing measures were necessary. Corneal cyanoacrylate gluing with nasal juxtalimbal conjunctival resection with bandage lens placement was carried out (see online suppl. materials for intraoperative surgical video). There was no descemetocele formation, corneal perforation, or secondary microbial keratitis. His visual acuities following the procedure were 6/9+1 (left) and 6/12-1 (right). His C-reactive protein reduced from 387 mg/L to 8.7 mg/L supporting GPA remission 3 months following presentation. He is currently on tapering oral prednisone with weekly methotrexate 20 mg maintenance therapy without evidence of recurrence. A 5-month progress anterior segment photo is shown in Figure 4. The patient remains under the care of ophthalmology, rheumatology, and urology outpatient departments at time of publishing.