Eye manifestations in patients with perinuclear antineutrophil cytoplasmic antibody-associated vasculitis: case series and literature review.
OBJECTIVE
To report and summarize eye manifestations of patients with perinuclear pattern antineutrophil cytoplasmic antibody [pANCA, myeloperoxidase (MPO)-ANCA]-associated vasculitis.


METHODS
The medical records of four consecutive patients with pANCA (MPO-ANCA) vasculitis who showed eye manifestations were retrospectively reviewed. In addition, the medical literature databases, PubMed and Japana Centra Revuo Medicina for Japanese literature, were searched for pANCA vasculitis patients with eye manifestations.


RESULTS
Three of the four patients treated at the Okayama University Hospital showed unilateral or bilateral scleritis. In the literature review, eight of the 27 patients showed ocular surface manifestations such as scleritis and peripheral keratitis. Other frequent eye presentations were posterior segment manifestations such as central or branch retinal vein occlusion, optic neuropathy, and acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Systemically, the most frequent manifestations were glomerulonephritis in the present patients as well as in the patients reported in the past studies.


CONCLUSIONS
Ocular surface manifestations and posterior segment manifestations were major eye presentations in patients with pANCA-associated vasculitis. ANCA testing including both pANCA and cytoplasmic pattern antineutrophil cytoplasmic antibody would help establish a systemic diagnosis in patients with eye manifestations such as scleritis, retinal vein occlusion, optic neuropathy, or APMPPE.
Matsuo
Toshihiko
T
Department of Ophthalmology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan. matsuot@cc.okayama-u.ac.jp
D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D005128:Eye Diseases / Q000209:etiology*; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D007634:Keratitis / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000209:etiology; D009195:Peroxidase / Q000097:blood*; D010857:Pigment Epithelium of Eye / Q000473:pathology; D012170:Retinal Vein Occlusion / Q000175:diagnosis / Q000209:etiology; D012189:Retrospective Studies; D015423:Scleritis / Q000175:diagnosis / Q000209:etiology; D014657:Vasculitis / Q000150:complications* / Q000276:immunology
D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase