Scleroderma or systemic sclerosis (SSc) is a chronic autoimmune connective with unknown etiology and poorly understood pathogenesis. Like other connective tissue diseases, SSC is more common in females, and the highest incidence is observed in the time frame from the third to the fifth decade. The striking array of autoimmune, vascular and fibrotic changes that develop in almost all patients makes SSc unique among connective tissue diseases. Although no animal model developed for SSc to date fully represents all features of human disease, some animal models that demonstrate features of SSc help to better understand the pathogenesis of the disease and develop new therapeutic options. Well-defined animal model selection is critical to the success of a well-conducted in vivo study. It includes induction methodology as well as a variety of approaches for studying skin fibrosis in these models, such as histopathology, immunohistochemistry, dermal thickness, hydroxyproline content of the skin, and flow cytometry analysis of dermal cells. Monitoring the model during the induction of the SSc mouse model, and determining the timing of treatment initiation is very important in the evaluation of treatment success. For this purpose, in vivo evaluation methods have an important place to observe the realization success of the model during induction in the animal. Evaluating the initial skin thickness of each mouse and the response created as a result of the induction, and showing how much response to the treatment according to the skin thickness at the beginning of the treatment is of critical importance for the success of the treatment. In this review, we aimed to evaluate skin fibrosis and lung involvement in a BLM-induced mouse model and to evaluate the differences between studies

Background/aim: To investigate the frequency and clinical relevance of an extended autoantibody (ab) profile in patients with SSc. Materials and Methods: In this cross-sectional study, serum from 100 consecutive patients was subjected to indirect immunofluorescence (HEp-20-10/primate liver mosaic) and Systemic Sclerosis Profile by EUROIMMUN (Lübeck, Germany) to evaluate ANA and autoantibodies against 13 different autoantibodies in patients with SSc less than three years. Results: 93 of 100 patients were positive for ANA by indirect immunofluorescence (IIF). The prevalence of Anti-Scl70 ab was 41%, anti-centromere (ACA) 27%, and anti-RNA polymerase (RNAPIII) 15%. Scl70 was more associated with diffuse subtype (p<0.001), ILD (p<0.001), and high mRSS (p=0.002); ACA with limited disease (p<0.001), less ILD (p<0.001), overlap (p=0.017) and low mRSS (p=0.024); RNAPIII with diffuse disease (p=0.027), ILD (p=0.016) and high mRSS (p=0.001). Fifty-three patients showed single positivity (26 anti-Scl70, 16 ACA, 6 anti-RNAPIII, 1 anti-Ku ab, 1 anti-PM/Scl100, 2 anti-PM/Scl75, 1 anti-Ro52), whereas 32 patients had multiple auto-antibody positivities. Among common SSc-specific autoantibodies, Scl70 and RNAPIII showed the highest co-occurrence (n=4). One patient was simultaneously positive for anti-RNAPIII ab and ACA, and one was positive for ACA and Scl70. The clinical features were not statistically different between single and multiple autoantibody-positivity for common SSc-specific autoantibodies (ACA, Scl70, and RNAP III), except for digital ulcer in multi-antibody positive ACA group (p=0.019). Conclusion: Based on our results, co-expression of auto-antibodies is not uncommon in SSc patients. Although SSc-specific auto-antibodies generally show known clinical features, the clinical presentation of the co-expression in specific and non-specific auto-antibody positivity continues to be important.