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Showing 2 results for Systemic Scleroderma
Statistical analysis of clinical and laboratory characteristics of systemic sclerosis
Elham Rajaei , Amin Hessam, Karim Mowla , Zeinab Deris Zayeri ,
Volume 77, Issue 9 (12-2019)
Abstract
Background: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by vasculopathy and excessive collagen deposition in the skin and internal organs. Based on the skin damage we divide the patients into two groups: limited and diffuse SSc. In restricted type, the thickness of the skin is limited in the face and distal parts of the elbows and knees, with less involvement of internal organs, whereas in diffuse type, facial skin, organs, and trunk generally increase in thickness and internal visceral involvement is noticeable as renal failure, pulmonary artery pressure, and interstitial lung fibrosis. According to the prevalence studies, the prevalence of systemic sclerosis is estimated to be 15 cases per 100,000, of which the prevalence in women is 3 times more than men. Based on clinical evidence the prevalence of SSc is high in Khuzestan province. The aim of this article was to evaluate the demographic features of SSc in Khuzestan province, Iran.
Methods: This cross-sectional study conducted on two hundred SSc patients referred to Rheumatology Clinic of the Golestan University Hospital in Iran, from 2001 to 2015. Patients were selected based on the American College of Rheumatology classification criteria for SSc and were divided into diffuse and restricted types based on the site of skin involvement. Individual characteristics and clinical symptoms were extracted from the information in the patient records which were carefully compiled by physicians. Data on age, sex, race, duration of illness, and family history of SSc were collected orally.
Results: 91.5% of the enrolled patients were female. The mean age was 44 years and the most common race in our study was Lor. The prevalence of the diffuse form of SSc was 68% and the limited form was 32%. The Raynaud's phenomenon was detected in 100% of patients. Sclerodactyly and interstitial lung disease were found in 88.5% and 54.5% of patients respectively. Laboratory features such as ANA (93%), Anti-Scl-70 (77.5%) and anti-centromere antibody (ACA) (70%) of SSc patients were reported.
Conclusion: According to the results of this article, the most systemic sclerosis patients in Khuzestan province were middle-aged Lor race females and the most common type was diffuse systemic sclerosis.

Association between atherosclerosis and parenchymal lung involvement in systemic sclerosis
Isa Khaheshi, Taraneh Faghihi Langroudi , Sima Salimi, Marjaneh Karimi, Abbas Arjmand Shabestari , Maral Edalati, Shahabeddin Gorji, Elham Mahmoudi ,
Volume 78, Issue 4 (7-2020)
Abstract
Background: Systemic sclerosis is an autoimmune disease affecting connective tissues, (including epidermal, subepidermal, microvasculature, etc.), leading to various extent of end-organ damage. The leading cause of mortality among these patients is lung involvement. The cardiovascular events happen more frequently in patients suffering systemic scleroderma, comparing to healthy population. This study was designed to clear the correlation between development of coronary calcification (as an indicator of atherosclerosis) and lung disease in these patients.
Methods: All patients with definite diagnosis of systemic sclerosis, who referred to Shahid Modarres Hospital between March 2011and March 2014, entered to the study. Patients suffering hypertension, hyperlipidemia and who had a past or current history of smoking were excluded from the study. Atherosclerosis was determined by coronary calcium score (based on Agatston score) and the severity and extent of lung disease was assessed by wells scoring system and Warrick scoring system (based on lung CT scan without contrast). The spearman correlation analysis was done on the data by SPSS software, version 20 (IBM SPSS, Armonk, NY, USA). All the patients had informed consent and no additive charge was delivered.
Results: The study population consisted of 25 patients with systemic sclerosis. 21 patients were female and the 4 other ones were male. The mean age was 67±4 years old. The mean Wells score in patients was 13±2.59, the mean Warrick severity score was 6.54±6.16 and the mean Warrick extent score was 14.42±14.59. No correlation was observed between presence of calcification in coronary arteries (Wells score r2=0.63  P=0.77, severity score r2=0.27, P=0.2, extent score r2=0.11, P=0.6), aorta annulus (Wells score r2=0.04, P=0.83, severity score r2=0.06, P=0.77, extent score r2=0.06,  P=0.76) and thoracic aorta (Wells score r2=0.05, P=0.83, severity score r2=0.03, P=0.9, extent score r2=0.03, P=0.9) with the severity and extent of lung involvement.
Conclusion: It seems that the presence of coronary atherosclerosis or calcifications in aorta annulus and thoracic aorta (assessed by Agatston calcium score) has no significant correlation with the severity and extent of lung disease (assessed by Wells and Warrick score) in patients with systemic sclerosis.

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