Isolated necrotizing scleritis revealing silent pulmonary granulomatosis with polyangiitis: A case report of rare diagnostic clue.
Nabaa Ihsan Awadh, Faiq I Gorial, Ahmed Dheyaa Al-Obaidi et al.
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Case in brief
A 31-year-old man with recurrent unilateral necrotizing anterior scleritis that resisted corticosteroids and immunosuppressants had no systemic symptoms. Testing showed mildly elevated anti-proteinase 3 antibodies (25 AU/mL), while chest CT unexpectedly identified a 5-cm solitary pulmonary mass. Biopsy showed necrotizing granulomatous inflammation, multinucleated giant cells, and vasculitis, confirming granulomatosis with polyangiitis (GPA). He received pulsed intravenous methylprednisolone and monthly cyclophosphamide, followed by rituximab maintenance. Ocular inflammation and visual function improved markedly, with no new systemic manifestations during follow-up.
What made this case unusual
Treatment-resistant necrotizing scleritis was the presenting clue to GPA despite absent systemic symptoms; evaluation uncovered a clinically silent solitary pulmonary mass, with biopsy establishing the diagnosis despite only mildly elevated anti-proteinase 3 antibodies.
Diagnostic / clinical pearl
In atypical or refractory necrotizing scleritis, consider systemic vasculitis even without systemic symptoms. When serologic findings are mild or nonspecific, tissue biopsy can be decisive.