Histopathology of the human retina in retinitis pigmentosa

Prog Retin Eye Res. 1998 Apr;17(2):175-205. doi: 10.1016/s1350-9462(97)00012-8.


The term, 'retinitis pigmentosa', refers to a heterogeneous group of inherited diseases that cause degeneration of rod and cone photoreceptors in the human retina. Loss of photoreceptor cells is usually followed by alterations in the retinal pigment epithelium and retinal glia. Ultimately, degenerative changes occur in the inner retinal neurons, blood vessels, and optic nerve head. This chapter provides background information on the genetics of retinitis pigmentosa and a summary of the histopathologic alterations in human retinas caused by this disease. Detailed information is provided on the effects of the primary disease process on the rod photoreceptors and changes in the other retinal components, all of which are important considerations for understanding and developing therapies for retinitis pigmentosa.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Astrocytes / pathology
  • Axons / pathology
  • Humans
  • Neuroglia / pathology
  • Optic Disk / pathology
  • Photoreceptor Cells / pathology
  • Retina / pathology*
  • Retinal Ganglion Cells / pathology
  • Retinal Vessels / pathology
  • Retinitis Pigmentosa / genetics
  • Retinitis Pigmentosa / pathology*