| Abstract | Senilna makularna degeneracija (engl. age-related macular degeneration - AMD) je vodeći uzrok sljepoće u zemljama razvijenog svijeta. AMD se, s obzirom na prisutnost koroidalne neovaskularizacije, dijeli na suhu i vlažnu formu bolesti. 80-85% slučajeva AMD-a otpada na suhu formu bolesti. Najčešće se pojavljuje u osoba starijih od 60 godina, bijele rase i europskog podrijetla. Predviđa se da će do 2040. godine broj oboljelih u svijetu doseći 288 milijuna. AMD je rezultat interakcije genetskih i okolišnih čimbenika rizika. Varijacije u CFH, CFB, C2 i HTRA1 genima predisponiraju osobu na razvoj bolesti. Starija dob, pušenje, kardiovaskularne bolesti te pretilost smatraju se čimbenicima rizika za razvoj AMD-a. Bolest započinje u retinalnom pigmentnom epitelu (RPE), a temeljni proces u podlozi je kronična autologna parainflamacija. Danas ne postoji općeprihvaćena definicija niti klasifikacija bolesti. Najčešće su u primjeni AREDS sustav klasifikacije u četiri kategorije te klinička klasifikacija predložena od strane Beckmanove inicijative za makularna istraživanja. Suhi AMD uzrokuje oštećenje centralnog vida praćeno gubitkom centralne vidne oštrine, pojavom centralnih skotoma, oštećenjem kolornog i stereoskopskog vida, otežanom adaptacijom na svjetlost i tamu te, u uznapredovaloj fazi bolesti poznatijoj kao geografska atrofija, otežano prepoznavanje lica. Temeljno kliničko obilježje suhog AMD-a je pojava druza na očnoj pozadini. Uz podrobnu osobnu i obiteljsku anamnezu te pregled očne pozadine, značajnu ulogu u postavljanju dijagnoze ima i optička koherentna tomografija, a u slučaju sumnje na konverziju suhe u vlažnu formu bolesti, važno mjesto zauzima i fluoresceinska angiografija. Trenutno nema odobrene terapije za suhu formu bolesti te se liječenje poglavito sastoji od primjene antioksidativnih pripravaka uključenih u AREDS2 formulu. Detaljnije poznavanje patofizioloških mehanizama potaknulo je provođenje više kliničkih ispitivanja u svrhu razvoja djelotvornih lijekova za suhi AMD. Ohrabrujuće rezultate pokazuju inhibitori sustava komplementa, posebno inhibitor C5 komponente komplementa, Zimura, koja je trenutno u fazi III kliničkog ispitivanja. |
| Abstract (english) | Age-related macular degeneration (AMD) is a leading cause of blindness in the developed world. With regard to the presence of choroidal neovascularization, AMD is divided into dry and wet forms. Dry form of AMD accounts for almost 80-85% of all diagnosed cases. It mostly affects the white population of European ancestry over the age of 60. It is expected that the number of persons with AMD worldwide could increase to 288 million in 2040. AMD is a result of interplay among genetic susceptibility and environmental risk factors. Genetic polymorphisms in CFH, CFB, C2 and HTRA1 genes predispose individuals to develop AMD. Older age, smoking, cardiovascular diseases and obesity are considered as risk factors for developing AMD. The disease starts in the retinal pigment epithelium with a chronic autologous parainflammation as the basic pathologic process underlying AMD. Currently there is no generally accepted definition or unified classification system for AMD. The most widely used classification systems are the AREDS system that classified AMD into four categories and the clinical classification proposed by the Beckman Initiative for Macular Research Classification Committee. Dry AMD leads to central vision impairment accompanied by the loss of visual acuity, appearance of central scotomas, color and stereo vision disturbances, poor light/dark adaptation and problems with face recognition in the late stage of dry AMD, which is also known as geographic atrophy. Drusen are key clinical hallmark of dry AMD. In addition to the detailed personal and family medical history and the dilated fundus examination, optical coherence tomography plays important role in the diagnosis of dry AMD together with fluorescein angiography when symptoms of conversion from dry to wet AMD are identified. At present there is no approved treatment for dry AMD and it mostly consists of AREDS2 antioxidants use. Better understanding of the pathophysiology of dry AMD has led to an increase in clinical trials designed to develop its effective treatment. Complement inhibitors are showing promising results, especially inhibitor of C5, also known as Zimura, which is undergoing phase III clinical trials. |
