Circular Perivascular Autofluorescence Pattern in Patients With Autoimmune Retinopathy

Abstract

Purpose: To report the characteristics and prevalence of a previously undescribed circular perivascular fundus autofluorescence (FAF) pattern in paraneoplastic and nonparaneoplastic autoimmune retinopathy. Methods: This retrospective case series used clinical and imaging data extracted from charts of patients with autoimmune retinopathy in whom FAF imaging was performed from the initial presentation to the last visit. Results: Six of 25 patients with autoimmune retinopathy and FAF imaging developed circular perivascular FAF changes. Three patients had paraneoplastic autoimmune retinopathy, and 3 had nonparaneoplastic autoimmune retinopathy. The lesions appeared a mean of 25 months after symptom onset; however, the timing varied from months to years and did not correlate with the overall disease course. The lesions were initially typically hyperautofluorescent and varied in progression, distribution, and quality. Optical coherence tomography showed hyperreflective subretinal deposits in the corresponding areas in most patients. Conclusions: To our knowledge, these are the first reported cases with this circular perivascular FAF pattern in nonparaneoplastic autoimmune retinopathy. This finding could also be a useful diagnostic imaging marker in some patients with autoimmune retinopathy.

Keywords: autoimmune retinopathy; chorioretinal scarring; imaging; posterior uveitis.

Figure 1.

Case 1: Fundus imaging showing initial findings, progression, and final findings. (A–D) At presentation, fundus autofluorescence (FAF) shows an area of nasal parafoveal hyperautofluorescence in the left eye. (E–H) Four months after symptom onset, the initial appearance of hyperautofluorescent circular lesions (arrows) is in a perivascular distribution. (I–L) Thirteen months after symptom onset, there is a progression in the number and confluency of the lesions in the same perivascular distribution. (M–P) Twenty-nine months after symptom onset, the circular perivascular lesions have become hypoautofluorescent. (Q) Montage color fundus photography shows that these lesions are difficult to appreciate on examination. Lines show the areas of the optical coherence tomography (OCT) scans in images S and T. (R) Lines through the hypoautofluorescent lesions correspond to the location of the OCT scans in images S and T. (S and T) OCT through the lesions shows hyperreflective subretinal foci. (U) Montage color fundus photography shows that these lesions are difficult to appreciate on examination. Lines show the areas of the OCT scans in images W and X. (V) Lines through the hypoautofluorescent and hyperautofluorescent lesions correspond to the location of the OCT scans in images W and X. (W and X) OCT through the lesions shows hyperreflective subretinal foci.

Figure 2.

Case 2: Fundus imaging showing initial findings, progression, and final findings. (A–D) Nine years after presentation, fundus photographs and the first documented fundus autofluorescence reveal hyperautofluorescent circular lesions (arrows). The circular scars are visible on the fundus photographs but are less obvious than on FAF. (E–H) Eleven years after presentation, the hyperautofluorescent circular lesions become more confluent and have expanded toward the posterior pole. (I–L) Twelve years after presentation, the hyperautofluorescent lesions continue to become more confluent and the posterior borders of the hyperautofluorescent areas have progressed farther toward the posterior pole (arrows). (M) Montage color fundus photography shows circular lesions in the periphery of the right eye. Lines show the areas of the optical coherence tomography (OCT) scans in images O and P. (N) Lines through the peripheral areas of hyperautofluorescence correspond to the location of the OCT scans in images O and P. (O and P) OCT images through the peripheral lesions show subretinal hyperreflective foci. (Q) Montage color fundus photography shows circular lesions in the periphery of the left eye. Lines show the areas of the OCT scans in images S and T. (R) Lines through the peripheral areas of hyperautofluorescence correspond to the location of the OCT scans in images S and T. (S and T) OCT images through the peripheral lesions show subretinal hyperreflective foci.

Figure 3.

Case 3: Fundus imaging showing initial findings, progression, and final findings. (A–D) At initial presentation, fundus photography and fundus autofluorescence are unremarkable. (E–H) Twenty-four months after presentation, hyperfluorescent circular lesions with a hypoautofluorescent center are present (black arrows). A hypoautofluorescent scar from previous syphilitic retinitis is visible superiorly (white arrow). (I–L) Sixty months after presentation, there was an increase in the size and number of hypoautofluorescent circular lesions in both eyes (arrows) and areas of the lesions are becoming confluently hypoautofluorescent along the vessels in both eyes. (M) Color fundus photography shows the superior, far peripheral area of scarring in the right eye. Lines show the areas of the optical coherence tomography (OCT) scans in images O and P. (N) Lines through the circular lesions with hypoautofluorescent centers and hyperautofluorescent rings correspond to the location of the OCT scans in images O and P. (O and P) OCT through the lesions shows the lesions to be partial-thickness, outer retinal hyperreflective lesions or full-thickness retinal hyperreflective lesions. (Q) Montage color fundus photography shows that the lesions are difficult to appreciate on examination. Lines show the areas of the OCT scans in images S and T. (R) Lines through the hypoautofluorescent lesions correspond to the location of the OCT scans in images S and T. (S and T) OCT through the lesions shows the lesions to be partial-thickness, outer retinal hyperreflective lesions or full-thickness retinal hyperreflective lesions.

Figure 4.

Case 4: Fundus imaging showing initial findings, progression, and final findings. (A–D) One month after symptom onset, fundus photography shows a longstanding chorioretinal scar inferonasal to the nerve in the left eye that predated the onset of cancer-associated retinopathy. Fundus autofluorescence (FAF) shows speckled hyperautofluorescent lesions concentrated around the arcades. (E–H) Four months after symptom onset, there is an increase in the lesion size and number and some circular lesions are becoming hypoautofluorescent on FAF. (I–L) Eight months after symptom onset, there is a progression in the number and confluency of the lesions, with the lesions becoming more predominantly hypoautofluorescent, although some hyperautofluorescent lesions are still seen. (M) Montage color fundus photography shows that these lesions are difficult to appreciate on examination. Lines show the areas of the optical coherence tomography (OCT) scans in images O and P. (N) Black lines through numerous hypoautofluorescent circular lesions correspond to the location of the OCT scans in images O and P. (O and P) OCT through the lesions shows hyperreflective subretinal foci. (Q) Montage color fundus photography shows that these lesions are difficult to appreciate on examination. Lines show the areas of the OCT scans in images S and T. (R) Lines through numerous hypoautofluorescent circular lesions correspond to the location of the OCT scans in images S and T. (S and T) OCT through the lesions shows hyperreflective subretinal foci.