PMID- 23385633
OWN - NLM
STAT- MEDLINE
DCOM- 20130617
LR  - 20240322
IS  - 1468-2079 (Electronic)
IS  - 0007-1161 (Print)
IS  - 0007-1161 (Linking)
VI  - 97
IP  - 5
DP  - 2013 May
TI  - Retinal pigment epithelial detachments and tears, and progressive retinal 
      degeneration in light chain deposition disease.
PG  - 627-31
LID - 10.1136/bjophthalmol-2012-302851 [doi]
AB  - BACKGROUND/PURPOSE: 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.
FAU - Spielberg, Leigh H
AU  - Spielberg LH
AD  - Department of Ophthalmology, Leuven University Hospital, Department of 
      Ophthalmology, 33 Kapucijnenvoer, Leuven 3000, Belgium. leigh.spielberg@gmail.com
FAU - Heckenlively, John R
AU  - Heckenlively JR
FAU - Leys, Anita M
AU  - Leys AM
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20130205
PL  - England
TA  - Br J Ophthalmol
JT  - The British journal of ophthalmology
JID - 0421041
RN  - 0 (Coloring Agents)
RN  - 0 (Immunoglobulin Light Chains)
RN  - IX6J1063HV (Indocyanine Green)
SB  - IM
MH  - Coloring Agents
MH  - Disease Progression
MH  - Female
MH  - Fluorescein Angiography
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Indocyanine Green
MH  - Male
MH  - Middle Aged
MH  - Paraproteinemias/*complications/metabolism
MH  - Retinal Degeneration/diagnosis/*etiology
MH  - Retinal Detachment/diagnosis/*etiology
MH  - Retinal Perforations/diagnosis/*etiology
MH  - Retinal Pigment Epithelium/pathology
MH  - Tomography, Optical Coherence
MH  - Visual Acuity/physiology
MH  - Visual Field Tests
MH  - Visual Fields/physiology
PMC - PMC3632992
EDAT- 2013/02/07 06:00
MHDA- 2013/06/19 06:00
CRDT- 2013/02/07 06:00
PHST- 2013/02/07 06:00 [entrez]
PHST- 2013/02/07 06:00 [pubmed]
PHST- 2013/06/19 06:00 [medline]
AID - bjophthalmol-2012-302851 [pii]
AID - 10.1136/bjophthalmol-2012-302851 [doi]
PST - ppublish
SO  - Br J Ophthalmol. 2013 May;97(5):627-31. doi: 10.1136/bjophthalmol-2012-302851. 
      Epub 2013 Feb 5.