Isolated necrotizing scleritis revealing silent pulmonary granulomatosis with polyangiitis: A case report of rare diagnostic clue.

A 31-year-old man presented with recurrent unilateral necrotizing anterior scleritis that was resistant to corticosteroids and immunosuppressants and occurred without systemic symptoms.

• Single-patient case report prepared in accordance with CAse REport (CARE) guidelines.
• The presentation had no ear, nose, and throat or renal involvement.
• Isolated necrotizing anterior scleritis as the sole initial manifestation of GPA is described as distinctly rare.

Serologic testing showed only mildly elevated anti-proteinase 3 (PR3) antibodies.

• Anti-PR3 antibodies were 25 AU/mL.
• The authors state that serologic findings were mild or nonspecific, so tissue biopsy was needed for diagnosis.

Chest CT unexpectedly revealed a clinically silent 5-cm solitary pulmonary mass.

• The patient had no systemic or respiratory symptoms.
• The mass was an incidental finding on chest CT performed during the systemic work-up of the refractory scleritis.

Histopathology confirmed GPA by showing necrotizing granulomatous inflammation with multinucleated giant cells and vasculitis.

• Biopsy of the pulmonary mass provided the definitive diagnosis despite only mildly elevated anti-PR3.
• The authors emphasize the critical role of tissue biopsy when serology is mild or nonspecific.

Treatment with pulsed IV methylprednisolone, then monthly cyclophosphamide, then rituximab maintenance produced marked improvement in ocular inflammation and visual function.

• No new systemic manifestations occurred during follow-up.
• The authors conclude that early multidisciplinary evaluation and timely rituximab therapy can prevent irreversible ocular damage and systemic progression.

This is described as the first documented Middle Eastern case of GPA presenting as isolated necrotizing scleritis with a silent pulmonary mass.

• The authors say the case adds educational value for ophthalmologists and rheumatologists.
• The case illustrates a rare diagnostic pathway from refractory scleritis to detection of silent pulmonary GPA.

Systemic vasculitis should be considered in atypical or refractory scleritis.

• Scleritis resistant to corticosteroids and immunosuppressants was the clue leading to the systemic work-up.
• This is based on a single-patient case report, so it does not provide generalizable frequency or outcome data.