Development of central retinal artery occlusion accompanied by choroidal folds in a patient with antineutrophil cytoplasmic antibody-associated vasculitis

Abstract

Rationale: We report a case of central retinal artery occlusion (CRAO) accompanied by choroidal folds in a patient positive for myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA). Patient concerns: The study involved a 67-year-old female patient who presented at the Department of Ophthalmology, Osaka Medical College, Takatsuki-City, Osaka, Japan on October 24, 2016 after becoming aware of a sudden decrease of visual acuity (VA) in her right eye. Other than suffering with scleritis 6-months previous, there was no obvious past history. Diagnosis: Upon examination, the VA in her right eye was hand motion, and the anterior segment of that eye showed thinning of the superior sclera. Macular edema in the inner retina and cherry red spots were observed in the ocular fundus, and optical coherence tomography (OCT) findings showed hyperreflectivity of the inner retina and choroidal folds. Fluorescein angiography (FA) examination of the fundus showed scattered areas of no retinal perfusion, and indocyanine green angiography (IA) findings of the fundus indicated a possible choroidal circulatory disturbance in her right eye. Blood test findings revealed the patient to be positive for MPO-ANCA. Based on the above findings, the patient was diagnosed with CRAO and choroidal circulatory disturbance due to ANCA-associated vasculitis. Interventions: For treatment, steroid semi-pulse therapy was initiated. Outcomes: Post treatment initiation, the fundus features and choroidal folds gradually improved, and her VA slightly improved to 0.08. Lessons: Based on the FA, IA, and OCT findings, the present case was considered to have CRAO accompanied by choroidal circulatory disturbance due to ANCA-associated vasculitis, a rare disease that may be complicated by choroidal circulatory disturbances.

Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Masquerading as Giant Cell Arteritis

“…During our literature review, we found several reported cases citing CRAO as the presenting symptom in patients with small vessel vasculitis, including eosinophilic GPA (9) and GPA (10).…”

Subclinical microvascular changes in ANCA-vasculitides: the role of optical coherence tomography angiography and nailfold capillaroscopy in the detection of disease-related damage

“…BVAS is a comprehensive multisystem clinical assessment in AAV and includes eyes in terms of scleritis/episcleritis, conjunctivitis/blepharitis/keratitis, and uveitis. Also, retinal changes are analyzed in BVAS as vasculitis, thrombosis, exudate, hemorrhages: however, all these abnormalities in pre-symptomatic phases can be undiagnosed and, thus, can lead to subclinical chronic damages, at the same time [ 22 , 25 , 26 ]. In accordance with our findings, the OCT-A can be considered as a key diagnostic tool in the early detection of possible subclinical retinal changes in AAV by adding information on disease-damage and activity in pre-symptomatic patients.…”

Subclinical microvascular changes in ANCA-vasculitides: the role of optical coherence tomography angiography and nailfold capillaroscopy in the detection of disease-related damage.

“…BVAS is a comprehensive multisystem clinical assessment in AAV and includes eyes in terms of scleritis/episcleritis, conjunctivitis/blepharitis/keratitis, and uveitis. Also, retinal changes are analyzed in BVAS as vasculitis, thrombosis, exudate, hemorrhages: however, all these abnormalities in pre-symptomatic phases can be undiagnosed and, thus, can lead to subclinical chronic damages, at the same time [22,[25][26]. In accordance with our ndings, the OCT-A can be considered as a key diagnostic tool in the early detection of possible subclinical retinal changes in AAV by adding information on disease-damage and activity in pre-symptomatic patients.…”

“…Posterior segment is less commonly involved in AAV, and abnormalities in retinal vessels and/or choroidal circulation have been rarely documented [14][15]. Only few case reports documented occlusive vasculitides in retinal network, particularly in ANCA negative AAV patients [16][17][18][19][20][21][22][23][24][25][26][27][28].…”