Introduction: Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a necrotising vasculitis affecting small and medium-sized vessels. It is a rare condition. Observation: This is a 40-year-old patient with a history of recurrent inflammatory episodes in both eyes of unknown cause, which have been progressing for a year. He consulted the ophthalmology department for eye pain, tearing and decreased visual acuity in both eyes, which had been progressing for two weeks. The initial ophthalmological examination revealed visual acuity of 1/50 in the left eye and “positive light perception” in the right eye. The diagnosis of bilateral peripheral ulcerative keratitis complicated by corneal perforation in the right eye was made. The patient was admitted to the ophthalmology department and an evisceration of the right eye was performed. In addition, an internal medicine consultation was requested to determine the cause. Paraclinical tests revealed a biological inflammatory syndrome with a C-reactive protein level of 20.15 mg/l; immunological tests were positive for ANCA with an anti-PR3 specificity of 182 AU/l (Antibody Unit/litre). The rest of the additional tests showed no abnormalities. Induction treatment was initiated with oral corticosteroid therapy (prednisolone 1 mg/kg/day) in combination with an immunosuppressant, namely methotrexate at a dosage of 0.3 mg/kg per week. The prognosis is favourable, with improved vision in the left eye and regression of the biological inflammatory syndrome. Conclusion: GPA is a serious condition that can affect visual prognosis in its purely ocular form. This atypical presentation should be consistently reported to raise awareness among clinicians about the early diagnosis of GPA.
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