One subset of patients with retinitis pigmentosa has immunologic defects.
Immunological evaluation of 20 patients with retinitis pigmentosa (RP) revealed significantly diminished circulating T-lymphocyte numbers and function and a high frequency of serum antibodies to human IgG in comparison with a healthy matched control population. However, these differences were due to severely abnormal results found in one subpopulation within the total patient group; this dichotomy was unrelated to the mode of inheritance or clinical severity of RP in individual patients. Thus, RP appears to be a syndrome rather than a disease and includes a form characterized by defective immunity. Whether this is involved in the pathogenesis of the retinal degenerative process is conjectural.
D000888:Antibodies, Anti-Idiotypic / Q000096:biosynthesis; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D007958:Leukocyte Count; D008213:Lymphocyte Activation; D008222:Lymphokines / Q000096:biosynthesis; D012174:Retinitis Pigmentosa / Q000145:classification / Q000276:immunology* / Q000503:physiopathology; D012397:Rosette Formation; D013601:T-Lymphocytes / Q000145:classification / Q000276:immunology* / Q000378:metabolism; D014794:Visual Fields
D000888:Antibodies, Anti-Idiotypic; D008222:Lymphokines