Recalcitrant infective scleritis masquerading an autoimmune necrotising scleritis: a primary presentation of biopsy-proven granulomatosis with polyangiitis

Abstract

Infectious scleritis is a rare but important cause of scleral inflammation. It is usually associated with an underlying ocular (prior ocular surgery or trauma) or systemic risk factor. A 53-year-old apparently systemically healthy woman presenting with spontaneous-onset pain, redness and watering in the left eye for 10 days was diagnosed with culture-proven Pseudomonas aeruginosa anterior scleritis. However, she was non-responsive to organism-sensitive antibiotics and scleral graft was performed twice, which showed graft re-infection. On repeated extensive systemic evaluations, the patient was diagnosed with biopsy-proven granulomatosis with polyangiitis (GPA). The patient was started on mycophenolate mofetil for both induction and maintenance phases and showed dramatic improvement with no recurrence till 1 year follow-up. High index of suspicion for autoimmune disorders, especially GPA, must be maintained for unilateral relentless infective scleritis masquerading as autoimmune necrotising scleritis. Mycophenolate mofetil holds a promising role for inducing as well as maintaining disease remission in ocular GPA.

Keywords: anterior chamber; ophthalmology.

Figure 1

Clinical photographs showing multifocal scleral abscess at presentation (A); status quo after 4 days of treatment (B); recurrent infection after first (C) and second (D) scleral grafts; resolution of scleral infection and inflammation after 8 weeks (E) and 1 year of starting mycophenolate mofetil (F).

Figure 2

Wedge-shaped hypodense lesion in the lower pole of right kidney (A); necrotising granulomatous inflammation involving tubulointerstitium and the surrounding capsule with focal vasculitis in the capsular region (B–D).