Tehran University Medical Journal

---

Systemic sclerosis is a generalized disorder of connective tissue, in which the pattern of disease extent, progression and outcome is heterogenous. To determine clinical features, disease extent and progression, we studied our patients in two phases of disease early (the first 3 years) and late phases (after 6 years of disease). 19 patients had diffuse cutaneous and 34 patients had limited cutaneous scleroderma. In patients with diffuse cutaneous scleroderma, disease progression has occurred mostly in the early phase of disease, but in patients with limited cutaneous scleroderma, disease progression was slow and incidious, so disease progression has occurred mostly in the late phase of the disease, thus raynaud's phenomenon, telangiectasia, hyperpigmentation and esophagitis were observed more in the late phase of the disease (statistically significant). In comparison of two groups, early and extensive organ involvement was observed in patients with diffuse cutaneous sclerodema.

---

Background: Systemic sclerosis (SSc) is an autoimmune rheumatic connective tissue disease. In normal wound healing process, fibroblasts are activated, proliferated and involved in tissue repair, and then removed by apoptosis. In systemic sclerosis, patient’s fibrosis occurs when fibroblasts become resistant to apoptosis and secrete a large amount of collagen and other extracellular matrixes. As the primary causes the disease are very complex and often unknown, it is necessary to consider or target the secondary causes of disease, such as the unresponsiveness of activated fibroblasts to apoptosis as the major factor in the creation and deployment of illness. In this study, we examined the expression levels of two key pro-apoptotic genes, Fas and Apaf-1, which are respectively involved in external and internal pathway of apoptosis.

Methods: In a case-control study skin biopsy samples were obtained from 19 patients with diffuse SSc, and 16 healthy controls. Dermal fibroblasts were cultured and total RNA was isolated from cell populations using High Pure RNA Isolation Kit (Roche Applied Science, Mannheim, Germany), followed by cDNA synthesis using RevertAid First Strand cDNA Synthesis Kit (Thermo Fisher Scientific Inc., Massachusetts, USA). Real-time PCR was performed using SYBRGreen gene expression master mix (Takara Shuzo, Co., Ltd, Shiga, Japan) and specific primers for Fas and Apaf-1. Real-time data were analyzed using the (2^-ΔCT)×1000 method. Statistical analysis was accomplished by using the SPSS software, v22 (IBM, Armonk, NY, USA). The P value less than 0.05 were recognized as a significant threshold. All data are represented as the mean ± SEM.

Results: Our results showed no significant difference in Fas (P=0.8) and Apaf-1 (P=0.17) mRNA expression levels between skin fibroblasts of systemic sclerosis patients and healthy controls.

Conclusion: In this study we observed no significant change in Apaf-1 and Fas mRNA levels in systemic sclerosis fibroblasts compared to control group. Hence, Apaf-1 and Fas are not transcriptionally activated in SSc fibroblasts. Further studies need to take place on protein levels and function of these proteins to confirm the mRNA transcription results.

---

Background: Systemic Sclerosis (SSc) is an autoimmune disease with multi-organ involvement mostly due to fibrosis and ectopic or excessive collagen fibers production in organs. Myocardial fibrosis is the main finding of cardiac involvement in patients with systemic sclerosis. In recent studies, the presence of Fragmented QRS complexes (FQRS) has been shown in the surface electrocardiogram in relation to fibrosis.
Methods: The present study is a case-control study during March 2019 to February 2020 that was conducted in 148 patients with scleroderma referred to the Rheumatology clinic in Shariati Hospital and 101 non-ischemic individuals in the control group matched by age and sex with the patient group. All the medical records were reviewed and those who were low risk according to 10-year atherosclerotic cardiovascular disease (ASCVD) risk assessment were selected as case groups. Data of ECG were evaluated for availability of FQRS or conductive abnormalities and calculating PR, QRS, QT, QTc and Tp-e intervals.