Acute posterior multifocal placoid pigment epitheliopathy and scleritis in a patient with pANCA-positive systemic vasculitis.

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.