Recalcitrant granulomatous sclerouveitis in a patient with granulomatous ANCA-associated vasculitis.

PURPOSE: To report an unusual case of granulomatous sclerochoroiditis.

DESIGN

Interventional case report.

METHODS

A patient with ANCA-associated granulomatous vasculitis presented with nodular necrotizing scleritis, which was recalcitrant to multiple systemic immunosuppressive therapies and progressed to a blind painful eye, which was enucleated.

RESULTS

Histopathology revealed extensive occlusive vasculitis, diffuse T- and B- cellular infiltration, and lymphoid granulomatous formation. Enhanced MHC class II antigens, adhesion molecules, and Fas (CD95) and FasL (CD95L) were detected in the lesion.

CONCLUSION

Granulomatous sclerochoroiditis with aggressive immune reaction can be a complication of ANCA-associated granulomatous vasculitis.