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Objective: Compared to the general population, malignancy is more common in patients with systemic sclerosis (SSc) and is one of the leading causes of mortality. The objective of this study was to assess the frequency and types of malignancies, and its association with SSc features. Methods: Medical records of 141 patients who were followed up in the Department of Rheumatology at Kocaeli University Medical Faculty Hospital and who met the 2013 American College of Rheumatology/European League Against Rheumatism SSC classification criteria were retrospectively analyzed. Clinical, demographic, laboratory and radiological information of the patients were obtained from the patient files. Results: Of the seven cases with malignancy, two had non-small cell lung cancer, two had breast cancer, one had larynx cancer, one had endometrium cancer, and one had chondrosarcoma. Apart from malignancy, a female patient was diagnosed with monoclonal gammopathy of undetermined significance. Four of the patients diagnosed with malignancy were evaluated as limited, and three with diffuse skin involvement. Modified Rodnan skin score was significantly higher in SSc patients with malignancy than in the patients without malignancy (p=0.045). No significant difference was found in terms of disease subtype, gender, age, auto-antibody and disease duration. Conclusion: Since the risk of malignancy is increased in patients with SSc, patients should be followed carefully. Malignancy can be diagnosed before the onset of SSc, concurrently or during the follow-up. SSc can be seen as a paraneoplastic syndrome, especially in patients with positive anti-RNAP-III antibody, and these patients should be monitored in terms of malignancy. [ABSTRACT FROM AUTHOR]

Objective: Quantitative Sensory testing (QST) is a non-invasive method used in the diagnosis and classification of peripheral neuropathies. This study aimed to evaluate peripheral small and large fibers involvement by QST in patients with systemic sclerosis and to evaluate its relationship with digital ulcer formation. Methods: Patients with systemic sclerosis who applied to the rheumatology unit between April and September 2013 were included in the study. All patients were evaluated with a detailed medical history and physical examination. Imaging and laboratory results and information on organ involvements were obtained from hospital files. Patients were evaluated with QST to determine large and small fibers involvement. Results: The study was conducted with 50 (92% female) systemic sclerosis patients. The mean age was 47±12 years, the mean disease duration was 11.3±7.2 years. Sixty-six percent (n=33) of the patients had a history of digital ulcer and/or pitting scar. In QST evaluation, 84% (n=42) of patients had abnormality in vibration and/or cold detection thresholds. The number of patients with upper extremity vibration detection threshold abnormality was higher than that of the patients with lower extremity abnormality (74% vs 40%). There was no statistically significant difference between the groups with and without QST abnormalities regarding the clinical features including digital ulcer/pitting scar history (p>0.05 for all). Conclusion: In this study, significant number of patients with systemic sclerosis had QST abnormality, which is evident in the vibration detection thresholds. Longitudinal studies in early systemic sclerosis patients are needed to determine the clinical importance of QST. [ABSTRACT FROM AUTHOR]

Objective: Digital ulcers related to systemic sclerosis (SS) are associated with disability and mortality in the progression of the disease. In our study, we investigated the duration of treatment and drug-related side effects on patients, who were started bosentan for the treatment of digital ulcers. Methods: SS patients with digital ulcers, who had a follow-up period in outpatient and inpatient clinics of Rheumatology Department, were included in our study. Duration of treatment and drug-related side effects were retrieved from the patient’s files. For the patients who did not come to the clinic in the last 6 months, visits were made over the phone. Results: A total of 30 patients were included in the study. The mean age of SS diagnosis was 37.5±14.7 years and 25 of these patients were female. The distribution of disease patterns was limited cutaneous for 25 patients and diffuse cutaneous for 5 patients. The median length of bosentan treatment was 31.0±23.9 months. Five patients required the discontinuation of bosentan due to adverse events including toxic hepatitis (2 patients) and headache (3 patients). Also, 3 patients discontinued the treatment on their own, despite the absence of adverse effects or toxicity. Conclusion: Bosentan, used for the treatment of digital ulcers in SS patients, has been shown to have an acceptable side-effect profile. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to determine the frequency and the causes of left ventricular systolic dysfunction (LVSD) in systemic sclerosis (SSc) patients. Methods: SSc patients admitted to the Rheumatology Department of Faculty of Medicine, Hacettepe University between January 2015 and February 2017 were included in the study. The echocardiographic results of the patients were evaluated retrospectively and a left ventricular ejection fraction (LVEF) of <50% was defined as LVSD. Demographic and clinical characteristics of patients with and without LVSD were compared. Results: Two hundred and sixty-two (female/male: 240/22) SSc patients were included. Diffuse SSc was present in 65 (24.8%) patients. Eight (3.1%) patients had LVSD. No significant difference was found between patients with and without LVSD in terms of gender, disease duration, disease type, autoantibody specifity and the frequency of interstitial lung disease and/or pulmonary arterial hypertension; however, patients with LVSD were older (p=0.02) and had coronary artery disease (CAD) more frequently (p<0.001). In three patients, the cause of LVSD was coronary artery disease, and the age at diagnosis of LVSD in these patients was higher than patients without CAD (p=0.03). Results of cardiac magnetic resonance imaging were available in 4 of SSc patients with LVSD and revealed myocardial involvement of SSc in 2 patients. Conclusion: In this study, the frequency of LVSD in SSc patients was consistent with the literature. Microvascular ischemia and CAD may play a role in the etiology of LVSD in patients with SSc and especially elderly patients should be evaluated carefully for the presence of CAD. [ABSTRACT FROM AUTHOR]

Objective: Our study aims to investigate and determine the sclerostin levels in blood and saliva of patients with scleroderma (systemıc sclerosis) and to compare their levels with those of healthy volunteers. In the literature, there is no sufficient number of studies that demonstrate the association between scleroderma and sclerostin. Sclerostin is thought to play a role in the pathogenesis of scleroderma. Material and Method: In our study, 35 scleroderma patients and 35 healty volunteers were enrolled. Blood and saliva sclerostin levels of the patient and healthy controls were measured. Results: The level of sclerostin in blood and saliva of the scleroderma group was measured to be significantly higher than in the control group (p <0,05). Conclusion: The level of sclerostin increases in scleroderma that is a chronic autoimmune disease. Sclerostin is an inhibitor of Wnt signaling pathway. These results may point that sclerostin or Wnt pathway have a role in the pathogenesis of scleroderma. However further studies involving more participants are needed in order to verify this hypothesis. [ABSTRACT FROM AUTHOR]

Bone destruction, which has an unknown etiology, can be seen in the course of systemic sclerosis. On the other hand, osteolysis may not always be acro-osteolysis. In this case report, a patient with systemic sclerosis and osteolysis in bilateral distal ulna is reported to emphasize the importance of being careful about the presence of osteolysis in tubular bones in patients with systemic sclerosis. Osteolysis in systemic sclerosis may be associated with disease severity. [ABSTRACT FROM AUTHOR]

Objective: Endothelial dysfunction and vasculopathy are crucial pathogenic factors in systemic sclerosis. Increased concentrations of plasma asymmetric dimethylarginine (ADMA) may also contribute to endothelial dysfunction in patients with systemic sclerosis. We evaluated the relationship between ADMA and aortic elastic properties in patients with systemic sclerosis. Material and Method: Plasma ADMA levels were measured in 30 patients with systemic sclerosis (28 females, mean age 40.7±11.6) and 30 healthy subjects (27 females, mean age 40.6±13). Aortic stiffness was determined with echocardiography. Results: In patients with systemic sclerosis, the mean value of ADMA was 0.53±0.13µmol/l compared with 0.46±0.15 µmol/l for control group. Systolic blood pressure, pulse pressure and aortic 'strain were found to be higher in patients with systemic sclerosis (p=0.027, p=0.048, p=0.037, respectively), diyastolic blood pressure and aortic distensibility was found lower in systemic sclerosis (p=0.039, p=0.045, respectively). There was no significant correlations between ADMA and aortic 'strain'. Conclusion: ADMA serum levels were increased in patients with systemic sclerosis, but no significant correlation was found between ADMA and aort's elastic structure (aortic 'strain', distensibility, and aortic stiffness index). [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to evaluate the relationship between the depressive symptoms with disease-related variables, functional status and quality of life in patients with systemic sclerosis (SSc). Material and methods: Forty patients diagnosed with SSc and 36 healthy control subjects were enrolled in the study. The demographic and clinical characteristics of the patients such as the Raynaud's phenomenon, SSc subtype, digital ulcers, gastrointestinal and lung involvement and disease activity were recorded. All patients were assessed using the Short Form-36 Quality of Life (SF-36 QoL) scale, the Health Assessment Questionnaire (HAQ) and the Beck Depression Inventory (BDI). Generalized pain and fatigue was assessed with the Visual Analogue Scale. Results: The patients with SSc had significantly higher scores in the depressive symptoms in terms of the BDI score compared to the healthy control group. According to the results of Spearman's analysis, there was a significantly correlation between the depressive symptoms with education level, generalized pain, dyspnea, disease activity score, gastroesophageal reflux, dysphagia, functional status, mental and physical score of the SF-36. Conclusion: Depressive symptoms is enhanced in patients with SSc. Depressive symptoms is especially associated with the generalised pain, disease activity, dysphagia, functional status, mental and quality of life in patients with SSc. [ABSTRACT FROM AUTHOR]

Objective: Autoantibody assays in sera have become more important in the diagnosis and follow up of autoimmune disease in recent years. Anti-centromere antibodies (ACA) in many autoimmune diseases including especially systemic sclerosis have been determined to be positive. The purpose of the study was to investigate the relation of antibody positivity in respect to autoimmune diseases. Methods: In this study, ACA in 4450 patients' serum samples which were suspected autoimmune disease sent to Selcuk University Meram Medical Faculty Clinical Microbiology Laboratory in 2009 were determined by the indirect immunofluorescence method and were evaluated qualitatively. Results: 38 (0.8%) of total 4450 patient's ACA are determined to be positive. 9 of patients with rheumatoid arthritis, 5 had arthritis, 5 had diseases not associated with autoimmunity, 3 had systemic sclerosis. 3 of 38 patients had Sjögren's syndrome, 3 had various viral infections, 3 had connective tissue disease, 2 had Raynoud disease, 2 had diabetes mellitus, 1 had iridocyclitis, 1 had vasculitis, 1 had celiac disease. Conclusion: The positivity of ACA has an important place in the diagnosis of autoimmune diseases. ACA is an important laboratory test for diagnosis and follow-up in many autoimmune diseases. [ABSTRACT FROM AUTHOR]

Pulmonary arterial hypertension (PAH) is an important complication of connective tissue diseases (CTD) and especially seen in systemic sclerosis, systemic lupus erythematosis (SLE), and mixed connective tissue disease (MCTD). In systemic sclerosis, PAH is isolated or accompanied by interstitial lung disease and currently, a major cause of mortality. It has been shown to be developed in approximately 10% of cases and annual screening with echocardiography has been recommended. Right heart catheterization is required for definite diagnosis. Limited skin involvement, late onset, Raynaud's phenomenon, digital ulcers, telangiectasias, diminished nail fold capillaries, antiU3RNP and anticentromere antibodies are known as risk factors for PAH development in systemic sclerosis. Following diffusion lung capacity for carbon monoxide (DLCO) and pro-brain natriuretic peptide (pro-BNP) levels can be helpful for evaluating PAH development. PAH in SLE linked to antiphospholipid antibodies and Raynaud's phenomenon in some studies. MCTD is an overlap syndrome with features of systemic sclerosis, SLE, polymyositis and positive anti-U1RNP antibodies. PAH develops in 9-27% of the patients and the leading cause of mortality in patients with MCTD. Endothelin receptor antagonists, prostacyclin analogs and phosphodiesterase 5 inhibitors are being used in patients with systemic sclerosis. In SLE/MCTD patients with early diagnosis immunosuppressive treatments may be effective. [ABSTRACT FROM AUTHOR]