Clinical Case In The Corneal Inflammation Spectrum In Anca Vasculitides

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare small-vessel necrotizing vasculitis. It can have serious organ-threatening implications making an accurate diagnosis and early treatment essential. This report describes a rare case of GPA diagnosis following the first presentation with ocular manifestations. It includes the multi-disciplinary management approach with a primary focus on the relative ocular sequelae.

University hospital, Ophthalmology department

Clinical case. A 65-year old male 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. Vision 6/5 (right) and 6/9 (left). Nodular scleritis extended to the right nasal limbus. The cornea was clear without defect. The anterior and posterior segments were normal. Systemic blood work and a punch biopsy of his 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.

Blood analysis demonstrated Anti-Neutrophil Cytoplasmic IgG Autoantibodies (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. His creatinine was 96 µmol/L with an estimated glomerular filtration rate of 71 ml/min/1.73m². Urine cytology demonstrated red blood cells, polymorphonuclear leucocytes without evidence of malignant cells. CT demonstrated lung, liver, prostate, nasal and ethmoidal/maxillary sinus involvement. Biopsies of both his right nasal sclera and nasal cavity mass demonstrated inflamed granulation tissue with multinucleate giant cells.

A diagnosis of granulomatous with polyangiitis was made, treated with intravenous methylprednisolone (IVMP) 1g for three consecutive days. He received two, 1g Rituximab infusions 14 days apart and cotrimoxazole 960mg 3 times-weekly. This case demonstrates the importance of recognising ocular manifestations of GPA as a first presentation of systemic vasculitis.