Purpose: To present acute posterior multifocal placoid pigment epitheliopathy and scleritis occurring in association with pANCA (perinuclear antineutrophil cytoplasmic antibody)-positive systemic vasculitis.
Design: Interventional case report.
Methods: A 69-year-old woman developed bilateral acute posterior multifocal placoid pigment epitheliopathy concurrent with rapidly progressive glomerulonephritis and bronchioalveolitis in both lungs.
Results: The patient showed a high titer of pANCA (myeloperoxidase-ANCA). She then developed scleritis in the left eye and multiple gastric ulcers.
Conclusion: Acute posterior multifocal placoid pigment epitheliopathy and scleritis in this patient are manifestations that are part of pANCA-positive systemic vasculitis. This association further supports that acute posterior multifocal placoid pigment epitheliopathy is caused by vasculitic inflammation of the choriocapillaris.