INCIDENCIJA, PREVALENCIJA I KLINIČKA OBILJEŽJA SISTEMSKE SKLEROZE U SPLITSKO-DALMATINSKOJ ŽUPANIJI

Dušanka Martinović Kaliterna, Mislav Radić, Ana Pavić

Systemic sclerosis (SSc) is a clinically heterogeneous generalized disorder which affects the connective tissue of the skin and internal organs such as gastrointestinal tract, lungs, heart and kidneys. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of collagen. Our aim was to to estimate the incidence, prevalence and disease characteristics of SSc in Split-Dalmatia County which has a population of 463,676 Caucasian people. A census of SSc cases for the period 2007-2009 was conducted using hospital records for case identification. Diagnoses were verifi ed by medical record review. All patients were evaluated by the validated American College of Rheumatology criteria for SSc. The exact 95% confi dence interval (CI) based on binomial distribution was created for the incidence and prevalence estimates. Results. Based on 51 verifi ed cases of SSc, prevalence was initially estimated to be 15.6 cases per 100,000 adults (95% CI 11.8-19.4), with an annual incidence of 3.2 new cases per 100,000 adults per year (95% CI 0.5- 4.1). The ratio of women to men was 5.2:1. The median age of patients was 55.1 yr. This study establishes baseline estimates of SSc occurrence and characteristics in a large Croatian cohort consisting of Causation people. These data should facilitate research regarding the role of geographic and environmental factors for this disease in comparison populations.

Sistemska skleroza (SSc) je multisistemska bolest nepoznatog uzroka karakterizirana fibrozom kože, krvnih žila i unutarnjih organa, uključujući gastrointestinalni trakt, pluća, srce i bubrege. Patogeneza SSc uključuje imunološke mehanizme, oštećenja krvožilja i aktivaciju fibroblasta s posljedičnim odlaganjem kolagena. Cilj ove studije bio je procijeniti incidenciju, prevalenciju i kliničke karakteristike sistemske skleroze (SSc) u Splitsko-dalmatinskoj županiji koja ima 463.676 stanovnika (uglavnom bijelci). Retrospektivnom studijom analizirana je bolnička dokumentacija u razdoblju od 2007. do 2009. godine te je učinjen popis SSc bolesnika. Konačna dijagnoza bolesti je potvrđena u skladu s kriterijima Američkog reumatološkog društva (engl. American College of Rheumatology). U svrhu procjene incidencije i prevalencije korištena je 95% granica pouzdanosti (CI, engl. Confi dence interval) zasnovana na binomnoj raspodjeli. Na temelju 51 SSc bolesnika procijenjena je prevalencija 15,6/100.000 (95% granica pouzdanosti [CI] 11,8-19,4) uz godišnju incidenciju od 3,2/100.000 novootkrivenih bolesnika. Omjer žena i muškaraca bio je 5,2:1. Medijan dobi bio je 55,1 godina života. Ovom studijom utvrđena je pojavnost i klinička obilježja SSc u Hrvatskoj. Potrebna su daljnja istraživanja kako bi se utvrdile moguće različitosti SSc u pojedinim dijelovima Hrvatske s obzirom na zemljopisne i okolišne čimbenike.