Showing 8 results for Scleroderma
R Najafizadeh , F Gharibduost , A Khalvat ,
Volume 58, Issue 2 (5-2000)
Abstract
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.
Jamshidi A.r , Gharib Doost F, Larijani B, Forouzesh Nia S M,
Volume 64, Issue 7 (8-2006)
Abstract
Background: Scleroderma is an important chronic disease with unknown ethiology and two subtypes: limited type: Skin involvement limited to distal of extremity and face. Diffuse type: Skin involvement is both distal and proximal of extremity, face and thrunk. Thyroid dysfunction is a main problem in these patients but there is no published data of Iranian scleroderma patients
Methods: This is a cross-sectional study to determine the prevalence of hypothyroidism (clinical, subclinical and autoimmune) in patients with scleroderma 125 patients with scleroderma selected and T3, T4, TSH Anti Tpo Ab and Anti TG Ab measured in them.
Results: 33 patients with scleroderma had hypothyroidism. (%26/4). Two patients with scleroderma had hyperthyroidism. (%1/6). %12/8 had clinical hypothyroidism. And %13/6 had subclinical hypothyroidism, %33/3 of patients with subclinical hypothyroidism and positive autoantibody had limited type. Where as %66/6 of patients with subclinical hypothyroidism and autoantibody had diffuse type, %28/5 of patients with clinical hypothyroidism and positive auto anti body had limited type. Where as %71/4 of patients with clinical hypothyroidism and auto anti body had diffuse type. All of patients with hyper thyroidism had diffuse type and autoantibody positive.
Conclusion: It seems hypothyroidism has an increased prevalence in patients with scleroderma and we suggest that thyroid function test must be done in primary evaluation of these patients
Aghaei M, Gharibdost F, Zayeni H, Akhlaghi M, Sedighi S, Rostamian Ar, Aghdami N, Shojaa M,
Volume 68, Issue 12 (3-2011)
Abstract
Background: Systemic scleroderma (SSc) is a generalized
connective tissue disorder of unknown origin which most notably is
characterized by skin thickening and organ damage. Endothelin-1
(ET-1) antibody plays a role in skin fibrosis. The
aim of this study was to determine the prevalence and correlation of different
manifestations of SSc with ET-1
plasma levels.
Methods: This cross-sectional analytical study was conducted on 95
patients (91 women and four men)
with scleroderma in 2006. The patients had
been referred to the Rheumatology Clinic of Shariati Hospital in Tehran, Iran.
The demographic data and signs and symptoms were entered in a questionnaire and
endothelin-1 concentrations were measured.
Results: The mean age of the patients was 38±12.29 years.
Diffuse cutaneous SSc (dcSSc)
was diagnosed in 52 and limited cutaneous
SSc (lcSSc)
in 43 patients. Raynaud's phenomenon (91%)
was the most common manifestation in the patients. The relationship between the
resorption of terminal phalanges due to fibrosis with the plasma concentration
of Endothelin-1 was statistically significant (p=0.001).
Pitting ulcers had significant relationships with endothelin-1 concentrations
too (p<0.05). No other significant relationships
were found between the other manifestation of the disease and Endothelin-1
concentration.
Conclusion: In
this study, Reynaud's phenomenon was the most frequent sign in patients with
scleroderma. Thus, it could serve as a tool for the diagnosis of scleroderma.
As there were no significant relationships between the other manifestations of
scleroderma with endothelin-1, a cohort study with a
larger sample size is suggested.
Elham Ahmadi , Sasan Fallahi , Behnoush Jalalian , Pouyan Amini Shakib ,
Volume 76, Issue 5 (8-2018)
Abstract
Background: Association of Addison's disease with connective tissue diseases such as scleroderma and Sjogren have been rarely reported. Anti-centromere antibody (ACA) has been associated with exocrine gland dysfunction in anti-Ro, anti-La negative Sjogren’s syndrome and may be one of the causes of xerostomia in community. The purpose of this article was to introduce a rare case of scleroderma-Sjogren intermediate phenotype with positive anti-centromere antibody in a known case of Addison’s disease admitted for dental caries and xerostomia.
Case Presentation: A 29-year-old woman with Addison’s disease referred to a dental clinic due to recurrent dental caries. Addison’s disease was confirmed by low basal serum cortisol level and unresponsive serum cortisol level to adrenocorticotropin hormone (rapid ACTH stimulation test). Signs of xerostomia, xerophthalmia, Raynaud’s phenomenon, gastro-esophageal reflux, masked face, osteoporosis, positive anti-centromere antibody, negative anti-Ro and anti-La antibodies and failure to match the pathology of the minor salivary gland of lip with Sjogren's disease were found. The diagnosis of scleroderma-Sjogren intermediate phenotype was raised with considering some of the symptoms of scleroderma and Sjogren and not the exact classification criteria for each of these two diseases. Hydroxychloroquine, fluoride and Biotene® mouthwash (Laclede, Inc., CA, USA) (oral moisturizing saliva), chewing gum containing xylitol plus artificial tear droplet was prescribed. Drinking plenty of fluids was recommended. Due to gastroesophageal reflux and osteoporosis, Pantoprazole and CinnoPar® (Cinnagen, Iran) (parathyroid hormone analogue) plus calcium and vitamin D supplements was administered. Regarding adrenal insufficiency, Prednisolone and Fludrocortisone were continued.
Conclusion: For evaluation of recurrent dental caries, especially in patients with autoimmune disease, anti-centromere antibody may be useful to identify the cause of dry mouth, as well as early detection of limited scleroderma or scleroderma-Sjogren intermediate phenotype.
Javad Moayedi , Zahra Musavi , Tayebeh Hashempour , Mohammad Ali Nazarinia , Behzad Dehghani , Zahra Hasanshahi ,
Volume 77, Issue 5 (8-2019)
Abstract
Background: Scleroderma is a chronic systemic disorder that affects the connective tissues. It is characterized by several immune manifestations, inflammation, vascular damage, and fibrosis. Some of the viral infections with complex mechanisms are involved in the development and progression of many autoimmune diseases, such as scleroderma. The present study aimed to investigate the serological prevalence of hepatitis B virus (HBV), hepatitis C virus (HCV), human immunodeficiency virus (HIV), Epstein-Barr virus (EBV), and cytomegalovirus (CMV) infections in Iranian patients with scleroderma.
Methods: In this descriptive study 65 patients with scleroderma and 65 healthy individuals who had no autoimmune diseases and matched for age and sex, from May 2017 to April 2018 at Shiraz HIV/AIDS Research Center, Shiraz University of Medical Sciences, Shiraz, Iran, were included. The serum of study participants were evaluated for cytomegalovirus specific immunoglobulin G (CMV-IgG), Epstein-Barr virus viral capsid antigen immunoglobulin G (EBV-VCA-IgG), hepatitis B surface antigen (HBsAg), hepatitis C virus antibody (HCVAb), and human immunodeficiency virus antibody (HIVAb) using commercially available the enzyme-linked immunosorbent assay (ELISA) kit.
Results: CMV-IgG was diagnosed in serum of all patients with scleroderma, while 49 (98%) healthy subjects had positive results for this test. In addition, EBV-VCA-IgG was diagnosed in 58 (89.2%) sclerodermic patients and 40 (80%) healthy subjects. The prevalence of CMV-IgG and EBV-VCA-IgG was not significantly different between patients and healthy subjects and had no significant relationship with age and sex. However, the titer of antibodies against CMV and EBV infections in the scleroderma group was higher than that in the control group (P<0.0001, and P<0.0001), respectively. The presence of HBsAg and HIVAb was not confirmed in any of the patients with scleroderma, but HCVAb was detected only in one patient. All of the individuals in control group were serologically negative for HBsAg, HCVAb, and HIVAb.
Conclusion: Serological prevalence of HBV, HCV, HIV, EBV, and CMV infections in patients with scleroderma is similar to the healthy group.
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.
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. |
Shamsi Zare, Payman Rezagholi,
Volume 80, Issue 7 (10-2022)
Abstract
Background: The incidence of adverse perinatal outcomes including increased risk of miscarriage, preeclampsia, preterm birth and stillbirth is higher in pregnant women with coronavirus. Pregnant women who are infected with the coronavirus have placentas that are abnormal compared to the placentas of healthy women. Examples of these adverse effects have been observed before and include reduced fetal growth, pre-eclampsia, premature birth and stillbirth. Scleroderma is an uncommon connective tissue disease and its most obvious manifestation is skin fibrosis. Patients may also have involvement of visceral organs, as a result, their digestive system, kidney and heart are affected. Scleroderma also exacerbates miscarriage, fetal growth retardation, intrauterine fetal death, and preterm delivery. Pregnant women with these problems need special measures, so this study was performed to report a successful cesarean section in a woman with coronavirus and scleroderma.
Case presentation: The patient was a 31-year-old pregnant woman with a gestational age of 29 weeks who presented to Sanandaj Besat Hospital in November 2021 with symptoms of shortness of breath and dyspnea. HRCT-positive, PCR-positive, bilateral pleural effusion, and pulmonary dilatation corona were diagnosed. Due to 3 liters of vaginal bleeding and diagnosis of Décollement 60% and severe preeclampsia underwent emergency cesarean section. The live baby was born weighing 1300 g with Apgar 7. During surgery, he received 3 units of FFB and 3 units of Cryoprecipitate. Microcalcifications and fibrin thrombi were reported in the pathology of intermittent nodules. The diagnosis and treatment of this patient has significant points that are mentioned below.
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Conclusion: Complications of pregnancy and childbirth in pregnant women infected with Corona virus include an increase in premature birth and an increase in the rate of cesarean section. Pregnancy in women with scleroderma at the right time and careful delivery monitoring will increase the probability of successful pregnancy outcome and all patients need counseling.
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