Thesis Type: Expertise In Medicine
Institution Of The Thesis: Gazi University, Tıp Fakültesi, Dahili Tıp Bilimleri, Turkey
Approval Date: 2016
Thesis Language: Turkish
Student: TUĞÇE EMİROĞLU
Co-Supervisor: MEHMET AKİF ÖZTÜRK, ABDURRAHMAN TUFAN
Open Archive Collection: AVESIS Open Access Collection
Abstract:Skleroderma is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Etiology and exact mechanism of disease is poorly understood. In the pathogenesis of the disease; vasculopathy (especially small vessels), cellular and humoral immunity (inflammation), visseral and vascular fibrosis are three main mechanisms. Scleroderma has a heterogeneous clinical picture and progression profile in affected individuals. Antimicrobial peptides (AMP) are composed of hundreds of peptid molecules that is present in all eukaryoticcells which are discovered recently. Human antimicrobial peptides are important molecules in innate immune system acting directly against pathogenic microorganisms. There are three main groups of antimicrobial peptides in humans; defensins, cathelicidin (LL37) and histatins. AMPs have protective roles against bacteria, viruses, fungiandcertain parasites. AMPs are profoundly expressed in body tissues that direct contact with microorganisms and exogenous harmful agents such as respiratory gastrointestinal and genitourinary epithelium and skin. They also exist in macrophages and neutrophil granules to contribute non-oxidative elimination ofphagocytized pathogens. AMPs have immunomodulatory features such as chemotaxis, opsonization, cytokin activation and antigene neutralization. A small number of studies have examined the role of AMPs on autoimmune diseases.. It has been demonstrated that the amount of alfa- and beta-2 defensin serum levels are increased in systemic lupus erythematosus patients. Likewise, the association between AMPs and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis has been reported.. No study investigated the role of AMPs on scleroderma patients. Hence, we aimed to investigate AMP serum levels and their possible association in these patients. The study is conducted in Gazi universtiy hospital, rheumatology outpatient clinic between 2015 and2016. Scleroderma patients were either newly diagnosed of on follow up visits for their disease. The demographic data, clinical features and involvements were recorded standardized forms during routine visits. Age- and sex- matched healthy individuals without an inflammatory disease were constituted the control group. Serum samples of subjects were studied for alfa defensin, beta-1, beta-2 defensinsand serum surfactane protein-A. There were 42 patients (40 female, mean age 42 years) and 38 healthy subjects (32 female, mean age 38 years) in the study. Defensin levels of patients were higher than control subject but it has not reached statistical significance. Serum surfactant protein A levels were higher in healthy subjects. There were no correlation between serum AMP levels and Rodnan score, PAB, FVC, FEV1, DLCO, sedimantation and CRP. there was an negative relationship between alfa defensin and CRP and positive correlation between ESR and beta-1 defensin. Serum antimicrobial peptides are affected by many environmental stimuli and have roles in many inflammatory disorders. To better elaborate contribution of these molecules on the pathogenesis of scleroderma further studies with detailed molecular tests are need.