Punctate inner choroidopathy (PIC) pic.jpg

Punctate inner choroidopathy (PIC) essex_f2.jpg

Punctate inner choroidopathy (PIC) 753741.fig.001.jpg

clinical characteristics

The clinical characteristics of a large series of patients with punctate inner choroidopathy (PIC) is presented in a new research report.

The term punctate inner choroidopathy (PIC) was first used by Watzke et al in 1984 to describe findings in a group of 10 patients with multifocal, well-circumscribed, usually small choroidal lesions. These lesions were said to variably evolve into hypopigmented scars, pigmented scars, or hyperplastic fibrotic scars as a result of choroidal neovascularization (CNV). Watzke and colleagues commented that the scars resembled those found in presumed ocular histoplasmosis syndrome (POHS). Histoplasmin skin test results, however, were negative in the 7 cases that underwent testing. In contrast to POHS, the evolution of PIC lesions was symptomatic and the disease tended to affect younger, myopic women.

Methods and Results

In a retrospective consecutive case series, patients seen during a 16-year period at Moorfields Eye Hospital who were diagnosed as having PIC and had a minimum of 12 months' follow-up were included. Patients were classified as having typical PIC or atypical PIC (larger, presumed ocular histoplasmosis syndrome–like lesions). Main outcome measures included development of choroidal neovascularization, development of new PIC lesions, and final visual acuity.
A total of 136 patients (271 eyes) were included. The average age was 32 years, 126 patients (93%) were female, and the mean refraction was –4.6 diopters. The overall mean follow-up was 6.2 years. Among 63 normal fellow eyes, 56 (88%) remained unchanged, 3 (5%) developed PIC lesions, and 4 (6%) developed choroidal neovascularization (CNV). Eyes with PIC lesions remained unchanged in 49 of 74 cases (66%), with 9 (12%) developing new PIC lesions and 16 (22%) developing choroidal neovascular membrane. In eyes with choroidal neovascularization, the mean visual acuity was 0.63 logMAR at study entry, 0.63 logMAR at 12 months, 0.61 logMAR at 2 years, and 0.71 logMAR at final review (mean, 6.1 years). Overall, 40 eyes with PIC-related choroidal neovascular membrane (26%) had final visual acuity less than 6/60. No differences were observed between typical and atypical PIC eyes in any of the outcome measures or in any of the subgroup analyses.

Disscussion and Conclusions

This study is the largest series to date of patients diagnosed as having PIC. The study includes all eyes with multifocal choroidal lesions in the absence of vitreous or anterior chamber inflammation (also in the absence of systemic disease known to cause choroiditis and of exposure to Histoplasma-endemic areas).

The researchers state that they could not identify any significant differences between eyes described as having typical PIC and those described as having atypical PIC. The researchers believe that typical and atypical PIC should be considered a spectrum of a single disease, PIC (a form of multifocal choroiditis), until differing etiologies can be demonstrated.

The investigators note that there is a substantial selection bias in the study population. Not only is there a referral bias to Moorfields Eye Hospital (the largest ophthalmic hospital in Europe), but 12 months of follow-up data were required for inclusion in the study, which likely would have excluded less severe cases such as asymptomatic eyes with normal vision not requiring ongoing care. It is likely, therefore, that the true prognosis in PIC is better than that described here. It is unlikely to be worse.

The observed clinical course of PIC was variable. Normal fellow eyes had an excellent prognosis, with only 1 losing substantial vision due to choroidal neovascularization (CNV). Twenty-two percent of eyes with PIC lesions went on to develop CNV. The PIC-related CNV had a somewhat aggressive course. Overall, 26% of eyes observed with PIC-related CNV had final VA less than 6/60. The PIC-related CNV does not, however, appear to be as aggressive as CNV in age-related macular degeneration — VAs in 43% of eyes remain at 6/12 or better.

It is interesting when observing the effects of CNV treatment on visual outcome to note that the final VA was similar in all groups with CNV regardless of the treatment they received. However, our study did not include any patients treated with anti–VEGF therapy. In the series by Chan et al (2007), the 4 patients with PIC treated with Avastin (bevacizumab) injections all improved, and none had VA worse than 6/12 at final review. Caution must be exercised, however, when using anti–VEGF agents in women of childbearing age.

Normal fellow eyes of patients receiving steroids were significantly more likely to develop CNV than those in patients without symptomatic choroiditis (19% vs 2%, respectively). The indication for the steroids was active PIC, and it was almost certainly the active disease rather than the steroid therapy causing the CNV. The inflammation is presumably the first step in the series of events that eventually leads to the development of CNV. The alternative explanation - that immunosuppression itself resulted in worse outcomes, possibly by exacerbating an underlying infectious cause - is unlikely.

In summary, the clinical outcomes of PIC are described. No differences could be identified between patients with typical and atypical PIC. The investigators believe these should be considered a spectrum of a single disease.