Elsevier

Survey of Ophthalmology

Volume 62, Issue 2, March–April 2017, Pages 113-126
Survey of Ophthalmology

Major review
Punctate inner choroidopathy: A review

https://doi.org/10.1016/j.survophthal.2016.10.003Get rights and content

Abstract

Punctate inner choroidopathy (PIC), an idiopathic inflammatory multifocal chorioretinopathy that predominantly affects young myopic women, appears to be relatively rare, but there are limited data to support accurate estimates of prevalence, and it is likely that the condition is underdiagnosed. The etiological relationship between PIC and other conditions within the “white dot syndromes” group remains uncertain. We, like others, would suggest that PIC and multifocal choroiditis with panuveitis represent a single disease process that is modified by host factors (including host immunoregulation) to cause the range of clinical phenotypes seen. The impact of PIC on the patient is highly variable, with outcome ranging from complete spontaneous recovery to bilateral severe sight loss. Detection and monitoring have been greatly facilitated by modern scanning techniques, especially optical coherence tomography and autofluorescence imaging and may be enhanced by coregistration of sequential images to detect change over time. Depending on the course of disease and nature of complications, appropriate treatment may range from observation to systemic immunosuppression and antiangiogenic therapies. PIC is a challenging condition where treatment has to be tailored to the patient's individual circumstances, the extent of disease, and the risk of progression.

Introduction

Punctate inner choroidopathy (PIC) is a relatively rare idiopathic inflammatory multifocal chorioretinopathy that most commonly affects young myopic women. Most of these lesions involve the posterior pole, arising at the level of the retinal pigment epithelium (RPE) and inner choroid in the absence of anterior chamber or vitreous inflammation (Fig. 1).86 Although it may be self-limiting with a favorable outcome, inflammation or neovascularization abutting the fovea may cause permanent visual loss. Depending on the course of disease and development of complications, treatment may range from observation to systemic immunosuppression and intravitreal antiangiogenic therapies.

PIC was first described by Watzke and colleagues in 1984 in a series of 10 young, otherwise healthy myopic women who presented with blurred central vision, photopsia, and paracentral scotomas and had well circumscribed yellow-gray lesions at the level of the inner choroid and retinal pigment epithelium associated with small neurosensory retinal detachments in the macula in the absence of detectable intraocular inflammation.86 Eight of the 10 patients presented with bilateral lesions, and choroidal neovascularization (CNV) developed in 6 patients. Although initially hypothesized to be secondary to myopia, the episodic nature of recurrences was subsequently acknowledged to be more suggestive of an underlying inflammatory pathogenesis.1

Punctate inner choroiditis, multifocal inner choroiditis.

Section snippets

Epidemiology and demographics

PIC is a relatively rare multifocal chorioretinopathy. It is difficult to make an accurate estimate of the incidence and prevalence of PIC for a number of reasons: first, there is a wide range of presentation and severity, such that many cases may remain unrecognized; second, there is uncertainty over its classification, notably whether it is a distinct entity from multifocal choroiditis with panuveitis (MCP) or part of the same spectrum; and third, in the absence of national registries or

Pathogenesis

The etiology of PIC is unknown, and its pathogenesis poorly understood. In common with the other inflammatory conditions that have been lumped together as “white dot syndromes,” it is proposed to be an autoimmune disease that arises in the context of polygenic susceptibility triggered by an environmental stimulus, such as infection, immunization, or stress. In the context of PIC, a plausible mechanism should ideally also explain the preponderance (but not exclusivity) of the female myopic

Clinical description

According to the survey by Gerstenblith and colleagues, initial symptoms in PIC are most commonly scotoma (91%), blurred vision (86%), photopsia (73%), floaters (69%), photophobia (69%), and metamorphopsia (65%). Loss of peripheral vision was reported in 26% of patients.30 These symptoms may fluctuate in severity, with 32% reporting symptoms waxing and waning before commencing treatment. Visual acuity at presentation is variable, reflecting the variable location of inflammatory lesions or their

Ancillary tests

Investigations used in diagnosing and monitoring PIC have mainly included fluorescein angiography, indocyanine green angiography, and OCT although newer modalities like fundus autofluorescence (FAF) imaging and OCT angiography are proving useful (Fig. 2, Fig. 3, Fig. 4).32, 40, 51, 79, 87, 90

The arteriovenous phase of fluorescein angiography shows punctate hyperfluorescent choroidal lesions with or without CNV. Areas of hyperfluorescence corresponding to the lesions will continue to persist

Management

The management of PIC is challenging for a number of reasons. First, the variable severity of disease between patients (or even in the same patient at different times) may mean that optimal treatment may appropriately range from observation to intensive immunosuppression or intravitreal anti-VEGF therapy. Second, any treatment strategy should distinguish between the major causes of sight loss in PIC. In pathogenetic terms, new or worsening PIC lesions are likely to reflect active inflammation,

PIC and pregnancy

Pregnancy has been associated with the development or recurrence of CNV, being reported secondary to PIC, POHS, or even in the absence of preexisting inflammation.66, 76 It is likely that this arises from elevated levels of angiogenic factors such as VEGF and placental growth factor. VEGF levels increase during the first trimester and then decline,50 whereas placental growth factor increases steadily throughout pregnancy.42

Many immune conditions improve during pregnancy as a result of

Prognosis

The visual outcome in PIC depends on the location of the lesions and whether they are complicated by the formation of CNV. Many patients do well, with visual acuity of 20/40 or better; however, approximately one-fifth will end up with a VA of <20/200 mainly from development of CNV and subretinal fibrosis.10, 59, 86 The risk of CNV is higher in PIC than other posterior and panuveitic conditions. The presence of active inflammation and previous CNV in the other eye increase the risk.6, 30, 62 The

Further research and patient engagement

Despite the advances in imaging that enable unparalleled visualization of the onset and development of PIC lesions, the etiology of PIC remains unknown, the indications for treatment are uncertain, and high-level evidence to support a treatment strategy is lacking. As discussed earlier, conducting randomized controlled trials of treatments for PIC is challenging; however, there is a need for this higher level evidence. We would urge the community of clinical experts and patients to work

Conclusions

In summary, PIC is a relatively rare, idiopathic inflammatory multifocal chorioretinopathy with a variable outcome ranging from complete spontaneous recovery to bilateral severe visual loss. Detection and monitoring have been greatly facilitated by modern scanning techniques, especially OCT and autofluorescence imaging, and may be enhanced by coregistration of sequential images to detect change over time. Our concern, however, is that even with these advances, there are many occasions when this

Method of literature search

The original literature search was undertaken in January 2016, with an updated search conducted in May 2016 to identify any “late-breaking” articles. The following databases were searched: Medline, EMBASE, and the Cochrane Library with a date limit of 1946 to the present for Medline, 1974 to present for EMBASE, and no date restriction for Cochrane. The search used the following terms: “Punctate inner choroidopathy,” “Punctate inner choroiditis,” and “multifocal inner choroiditis.” Papers were

Disclosures

The authors declare no conflicts of interest.

Acknowledgments

Pearse A. Keane has received a proportion of his funding from the Department of Health's NIHR Biomedical Research Centre for Ophthalmology at Moorfields Eye Hospital and UCL Institute of Ophthalmology. The views expressed in the publication are those of the author and not necessarily those of the Department of Health.

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