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ABSTRACT.Purpose: To report long-term follow-up of patients with pigmented paravenous retinochoroidal atrophy (PPRCA) and to assess the involvement of the choroid and retinal pigment epithelium (RPE) in PPRCA. Methods: Clinical features of PPRCA were studied retrospectively in four patients followed for 6-26 years. Retinal pigment epithelium and choroidal changes were analysed with fluorescein and indocyanine green (ICG) angiography. Results: The two younger patients, aged 16 and 28 years and followed for 6 and 18 years, respectively, showed stationary RPE atrophy and pigmentation. Indocyanine green angiography visualized slight to modest atrophy of the choriocapillaris. The two older patients, aged 69 and 70 years and each followed for 26 years, showed slow progression of disease during follow-up. Indocyanine green angiography revealed choriocapillaris atrophy partly extending into the areas shown as hyperfluorescent in fluorescein angiography. Conclusion: Pigmented paravenous retinochoroidal atrophy is probably a slowly progressive disease, particularly in older patients. The choriocapillaris atrophy in this disease is more properly evaluated by ICG angiography and can be underestimated by fluorescein anigiography.

There is considerable variability in functional manifestations among patients with crystalline retinopathy, even in intrafamilial cases.

Patients with retinitis pigmentosa and a group of controls were tested for their cellular immune response toward two retinal proteins, S-antigen and interphotoreceptor retinoid-binding protein (IRBP), as well as their reaction against two synthetic peptides ("M" and "N") derived from the sequence of S-antigen and peptide "R14", derived from IRBP. Positive responses to the retinal antigens were found in larger proportions and with higher levels in the patient group than in the controls. The difference between the two groups was statistically significant in their response to S-antigen, but the patients reacted better than the controls against the other antigens as well. Of particular interest was the finding that several patients responded to both retinal proteins and/or to their peptides. These patients suffered from severe retinal changes and the data are thus interpreted as suggesting that the responses to the retinal antigens are secondary to these changes and to nonphysiological release of retinal antigens.

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