title: Retinal pigment epithelial detachments and tears, and progressive retinal degeneration in light chain deposition disease.
abstract: OBJECTIVE
  Light-chain deposition disease (LCDD) is a rare condition characterised by deposition of monoclonal immunoglobulin light chains (LCs) in tissues, resulting in varying degrees of organ dysfunction. This study reports the characteristic clinical ocular findings seen in advanced LCDD upon development of ocular fundus changes. This is the first report to describe this entity in vivo in a series of patients.
  
  
  METHODS
  A case series of ocular fundus changes in three patients with kidney biopsy-proven LCDD. All patients underwent best corrected visual acuity (BCVA) exam, perimetry, colour fundus photography and fluorescein angiography; two patients underwent indocyanine green angiography, optical coherence tomography, ultrasound and electroretinography; and one patient underwent fundus autofluorescence.
  
  
  RESULTS
  Three patients, 53-60 years old at initial presentation, were studied. All three presented with night blindness, poor dark adaptation, metamorphopsia and visual loss. Examination revealed serous and serohaemorrhagic detachments, multiple retinal pigment epithelial (RPE) tears, diffuse RPE degeneration and progressive fibrotic changes. Neither choroidal neovascularisation nor other vascular abnormalities were present. Final best corrected visual acuity (BCVA) ranged from 20/40 to 20/300.
  
  
  CONCLUSIONS
  Progressive LC deposition in the fundus seems to damage RPE pump function with flow disturbance between choroid and retina. This pathogenesis can explain the evolution to RPE detachments and subsequent rips and progressive retinal malfunction.
authors: Department of Ophthalmology, Leuven University Hospital, Department of Ophthalmology, 33 Kapucijnenvoer, Leuven 3000, Belgium. leigh.spielberg@gmail.com
authors: Leigh H
authors: LH
authors: Spielberg
authors: John R
authors: JR
authors: Heckenlively
authors: Anita M
authors: AM
authors: Leys
mesh_terms: D004396:Coloring Agents; D018450:Disease Progression; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D007208:Indocyanine Green; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications* / Q000378:metabolism; D012162:Retinal Degeneration / Q000175:diagnosis / Q000209:etiology*; D012163:Retinal Detachment / Q000175:diagnosis / Q000209:etiology*; D012167:Retinal Perforations / Q000175:diagnosis / Q000209:etiology*; D055213:Retinal Pigment Epithelium / Q000473:pathology; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology; D058609:Visual Field Tests; D014794:Visual Fields / Q000502:physiology
chemical_list: D004396:Coloring Agents; D007147:Immunoglobulin Light Chains; D007208:Indocyanine Green
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