Punctate Inner Choroidopathy Inflammatory Signs: Prednisone Response Varies

For patients with punctate inner choroidopathy (PIC), two inflammatory signs observable with multimodal imaging (MMI) show differential response to treatment with systemic corticosteroids. Focal inflammatory lesions that split the retinal pigment epithelium/Bruch’s membrane (RPE/BrM) complex appear to show a rapid response; however, diffuse transient areas of outer retinal disruption (ORD) appear to show little response, according to findings published in Retina.

Prior treatment protocols for PIC have included antivirals, systemic glucocorticosteroids, intravitreal and sub-Tenon’s steroid injections, intravitreal anti-vascular endothelial growth factor (anti-VEGF) agents, and steroid-sparing immunosuppression. However, reports on treatment response did not delineate between inflammatory signs. Therefore, study investigators sought to use MMI in this retrospective, observational case series to describe the differential treatment response of two distinct PIC inflammatory signs.

Four eyes of four women with myopia (mean age 35 years, range 31-42) underwent a complete examination at either the East Bay Retina Consultants Inc. in Oakland, California or the Vitreous Retina Macula Consultants of New York, New York. Examinations included indirect ophthalmoscopy, slit-lamp biomicroscopy, and MMI (confocal fundus photography, color, and multicolor).

All four study eyes had two distinct inflammatory signs of active PIC: (1) acute focal hyperreflective lesions that split the RPE/BrM complex on optical coherence tomography (OCT), which on fundus autofluorescence (FAF) appeared hypoautofluorescent; and (2) more diffuse areas of ORD limited to the interdigitation zone and ellipsoid zone on OCT and corresponding to FAF hyperautofluorescence. Once all were given a diagnosis of noninfectious, idiopathic PIC, each was treated with oral prednisone, and response was followed with MMI.

All four study eyes showed a swift regression of RPE/BRM complex lesions (three participant eyes after one week, one eye after two weeks), but with a concomitant, paradoxical centrifugal ORD expansion. Eventually, the ORD did resolve in all eyes (mean of six weeks, range four to 10).

The study’s findings were limited by the small number of participants and the rarity of the disease, making conclusive assumptions about treatment difficult to make. Nevertheless, study investigators conclude that they were able to describe, “the unique multimodal imaging appearance of classic RPE/BrM complex PIC lesions preceding the onset of transient spots and zonal outer retinal photoreceptor disruption involving the EZ/IZ, suggestive of two interrelated but distinct inflammatory processes. The differential response to systemic glucocorticosteroids suggests the mechanisms of inflammation are distinct, but the original insult to the RPE and BrM may lead to the ORD due to exposure of the outer retinal photoreceptor antigens to the immune system.”

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.

Reference

Abdelhakim AH, Yannuzzi LA, Freund KB, Jung JJ. Differential Response to Glucocorticoid Immunosuppression of Two Distinct Inflammatory Signs Associated with Punctate Inner Choroidopathy.  Retina. [published online, August 12, 2020]. doi:10.1097/IAE.0000000000002950