Immune complexes in retinitis pigmentosa

García-Calderón, P A; Engel, Pablo; Cols, N.; García, Cosme

doi:10.3109/13816818409006122

Cited by 3 publications

(1 citation statement)

References 5 publications

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“…Recent studies by Kyger et al, has shown the presence of pathogenic auto-antibodies and T cell activation against retinal antigens in Royal College of Surgeons (RCS) rats [ 23 ]. Further, the presence of retinal auto-antibodies in RP patient’s blood has been reported [ 24 ]. Since the generation of IgG [ 25 ] producing B cells requires antigen specific T cell activation [ 26 ], the nature T cell response is of keen interest.…”

Section: Discussionmentioning

confidence: 99%

Immunological consequences of compromised ocular immune privilege accelerate retinal degeneration in retinitis pigmentosa

Mohan

Mishra

Muniyasamy

et al. 2022

Orphanet J Rare Dis

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Background Retinitis pigmentosa (RP) is a hereditary retinal disease which leads to visual impairment. The onset and progression of RP has physiological consequences that affects the ocular environment. Some of the key non-genetic factors which hasten the retinal degeneration in RP include oxidative stress, hypoxia and ocular inflammation. In this study, we investigated the status of the ocular immune privilege during retinal degeneration and the effect of ocular immune changes on the peripheral immune system in RP. We assessed the peripheral blood mononuclear cell stimulation by retinal antigens and their immune response status in RP patients. Subsequently, we examined alterations in ocular immune privilege machineries which may contribute to ocular inflammation and disease progression in rd1 mouse model. Results In RP patients, we observed a suppressed anti-inflammatory response to self-retinal antigens, thereby indicating a deviated response to self-antigens. The ocular milieu in rd1 mouse model indicated a significant decrease in immune suppressive ligands and cytokine TGF-B1, and higher pro-inflammatory ocular protein levels. Further, blood–retinal-barrier breakdown due to decrease in the expression of tight junction proteins was observed. The retinal breach potentiated pro-inflammatory peripheral immune activation against retinal antigens and caused infiltration of the peripheral immune cells into the ocular tissue. Conclusions Our studies with RP patients and rd1 mouse model suggest that immunological consequences in RP is a contributing factor in the progression of retinal degeneration. The ocular inflammation in the RP alters the ocular immune privilege mechanisms and peripheral immune response. These aberrations in turn create an auto-reactive immune environment and accelerate retinal degeneration.

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