Cause:Genetic ocular extracellular matrix protein mutations, esp of fibulin (Nejm 2004;351:320, 346)
Pathophys:Atrophic (dry) (90%) and neovascular (wet/exudative) (10%) forms; the latter causes 90% of the severe vision loss. Perhaps an inflammatory element since CRP elevated (Jama 2004;291:704)
Most common cause of severe visual loss in developed countries; present in 30%, 7% symptomatic prevalence over age 75 yr. Assoc w smoking (incid incr × 2.4—Jama 1996;276:1141, 1147); obesity; hyperlipidemia; positive fam hx; low antioxidant vitamins and zinc intake; Caucasians
Sx:Decr visual acuity
Si:Drüsen >63 µM, retinal pigment epithelial atrophy or clumping in macula; loss of central but preservation of peripheral vision
Prevent perhaps w vitamins C, E, ß-carotene, and/or zinc in normal doses (Jama 2005;294:3101); statins may protect (BMJ 2001;323:375 vs Jama ibid), perhaps vit D (Arch Opthamol 2011;129:481)
Laser rx of neovascular lesions
Photodynamic rx w verteporfin (Visadyne) (Med Let 2000;42:81)
Ocular injections w anti-angiogenesis drugs (Med Let 2006;48:85; 2005;47:55; Nejm 2006;355:1409,1419, 1432, 1493; 2004;351:2805, 2863); all off-label but Medicare covers; used for diabetic neovascular disease as well: