Clinical and Laboratory Characteristics and Mortality in Korean Patients with Systemic Sclerosis: A Nationwide Multicenter Retrospective Cohort Study

2018 ◽  
Vol 45 (9) ◽  
pp. 1281-1288 ◽  
Author(s):  
Ki Won Moon ◽  
Shin-Seok Lee ◽  
Yun Jong Lee ◽  
Jae-Bum Jun ◽  
Su-Jin Yoo ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Survival Rates ◽  
Clinical Manifestations ◽  
Geographic Area ◽  
Age At Diagnosis ◽  
University Hospitals ◽  
Anticentromere Antibody ◽  
Korean Patients ◽  
Common Diagnosis ◽  
Antibody Positivity

Objective.We aimed to investigate demographic and clinical features and predictors of mortality in Korean patients with systemic sclerosis (SSc).Methods.We performed a retrospective multicenter medical chart review in Korean patients diagnosed with SSc from 1986 to 2016 at 11 university hospitals representing each geographic area of Korea. SSc patients were defined according to the American College of Rheumatology preliminary classification criteria and subtyped as limited cutaneous (lcSSc) or diffuse cutaneous (dcSSc) SSc.Results.We enrolled 751 patients (female, 86.7%; mean age at diagnosis, 48.9 yrs). The most common organ involvement was interstitial lung disease (52.7%), followed by gastroesophageal reflux disease (32.9%) and pulmonary arterial hypertension (13.6%). Patients with lcSSc were more common than those with dcSSc (64.8 vs 35.2%), whereas anti-Scl-70 and anticentromere antibody positivity were identified in 302 (42.5%) and 175 (25.5%) patients, respectively. In the 46 (6.1%) patients who developed a malignancy, lung cancer (23.9%) was the most common diagnosis, followed by gastric (13%) and breast cancer (13%). During the study period, 57 (7.6%) patients died, and the 5- and 10-year survival rates were 94% and 87%, respectively. Increased age at diagnosis, cardiovascular involvement, and anti-Scl-70 antibody positivity were significant predictors of death.Conclusion.Clinical manifestations and survival rates in Korean SSc patients are similar to those of other populations. However, the prevalence of anti-Scl-70 antibody is higher in Korean SSc patients compared with whites, while the prevalence of anticentromere antibody is lower.

scholarly journals SAT0316 ANTI-PM/SCL ANTIBODIES IN SYSTEMIC SCLEROSIS: CLINICAL ASSOCIATIONS IN THE RESCLE COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1103.1-1104
Author(s):  
N. Iniesta-Arandia ◽  
G. Espinosa ◽  
A. Guillen del Castillo ◽  
C. Tolosa ◽  
G. M. Lledó ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Survival Data ◽  
Death Rate ◽  
Administrative Support ◽  
Medical Science ◽  
Survival Rates ◽  
Clinical Manifestations ◽  
Study Group ◽  
Allergy Immunol ◽  
Science Service

Background:Anti-PM/Scl antibodies are associated to systemic sclerosis (SSc) but are not specific to SSc. The true prevalence of anti-PM/Scl antibodies in SSc is unknown, ranging from 2.5% to 12.5%. An association between anti-PM/Scl antibodies with muscular involvement, pulmonary fibrosis, calcinosis, and a relatively benign prognosis have been described.Objectives:To compare the clinical manifestations and prognosis of SSc patients according the presence of anti-PM/Scl antibodies in the cohort of RESCLE (Spanish Scleroderma Registry).Methods:From the Spanish Scleroderma Study Group database, we selected patients in whom anti-PM/Scl antibodies had been tested. We compared demographic features, clinical manifestations, laboratory characteristics, and survival data between patients according the anti-PM/Scl antibodies status.Results:72 out of 947 (7%) patients tested positive for anti-PM/Scl antibodies. As presenting SSc manifestations, patients with anti-PM/Scl antibodies had higher prevalence of puffy fingers (11% versus 2%; p=0.002) and arthralgias (11% versus 4%; p=0.03), and lower prevalence of Raynaud’s phenomenon (65% versus 82%, p=0.002). Regarding cumulative manifestations, myositis (51% versus 15%; p<0.001), arthritis (43% versus 22%; p=0.001), and interstitial lung disease (ILD) (60% versus 45%, p=0.014) were more prevalent in patients with anti-PM/Scl antibodies. In fact, those patients with anti-Pm/Scl antibodies presented with FVC (77.4% ± 23.1% versus 85.8% ± 23,1%; p=0.006) and more severe ILD defined as FVC <70% (41% versus 24%; p=0.004). Death rate was similar in patients with and without PM/Scl antibodies (18% versus 17%; p=0.871).We did not find differences in terms of death rate nor in the causes of death (SSc and non-SSc related) according to the anti-PM/Scl antibodies profile.The 5- and 10-years survival rates of patients with anti-PM/Scl antibodies were 91% and 82% respectively, without differences with those without these antibodies (93% and 85%, respectively).Conclusion:In Spanish SSc patients, the presence of anti-PM/Scl antibodies confer a distinctive clinical profile. However, anti-PM/Scl antibodies do not play a role in the prognosis of these patients.References:[1]Stochmal A, Czuwara J, Trojanowska M, Rudnicka L. Antinuclear antibodies in systemic sclerosis: an update. Clin Rev Allergy Immunol 2020;58(1):40-51. doi: 10.1007/s12016-018-8718-8.Acknowledgments:We gratefully acknowledge all investigators who are part of the RESCLE Registry. We also thank the RESCLE Registry Coordinating Centre, S&H Medical Science Service, for their quality control data, logistic and administrative support and Prof. Salvador Ortiz, Universidad Autónoma de Madrid and Statistical Advisor S&H Medical Science Service for the statistical analysis of the data presented in this paper.Disclosure of InterestsNerea Iniesta-Arandia: None declared, Gerard Espinosa Speakers bureau: Glaxo-Smith-Kline, Janssen, Boehringer, Rovi, Alfredo Guillen del Castillo: None declared, Carles Tolosa Consultant of: Actelion pharmaceuticals, GSK, MSD., Gema Maria Lledó: None declared, Dolores Colunga Argüelles Consultant of: Actelion pharmaceuticals, GSK, MSD., Cristina González-Echávarri: None declared, Luis Sáez-Comet: None declared, Norberto Ortego: None declared, Jose Antonio Vargas-Hitos: None declared, Manuel Rubio-Rivas: None declared, Mayka Freire: None declared, Juan José Rios: None declared, Monica Rodriguez-Carballeira: None declared, Luis Trapiella Martínez: None declared, Vicent Fonollosa Pla Speakers bureau: Actelion, Carmen Pilar Simeón-Aznar Consultant of: Actelion pharmaceuticals, GSK, MSD., on behalf of RESCLE Investigators, Autoimmune Diseases Study Group (GEAS): None declared

scholarly journals Reassessing the Role of the Active TGF-β1 as a Biomarker in Systemic Sclerosis: Association of Serum Levels with Clinical Manifestations

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Andréa Tavares Dantas ◽  
Sayonara Maria Calado Gonçalves ◽  
Anderson Rodrigues de Almeida ◽  
Rafaela Silva Guimarães Gonçalves ◽  
Maria Clara Pinheiro Duarte Sampaio ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Vascular Involvement ◽  
Digital Ulcers ◽  
Serum Samples ◽  
Peripheral Blood Mononuclear ◽  
Pbmc Culture ◽  
Significant Difference ◽  
Skin Biopsies

Objective. To determine active TGF-β1 (aTGF-β1) levels in serum, skin, and peripheral blood mononuclear cell (PBMC) culture supernatants and to understand their associations with clinical parameters in systemic sclerosis (SSc) patients.Methods. We evaluated serum samples from 56 SSc patients and 24 healthy controls (HC). In 20 SSc patients, we quantified spontaneous or anti-CD3/CD28 stimulated production of aTGF-β1 by PBMC. The aTGF-β1 levels were measured by ELISA. Skin biopsies were obtained from 13 SSc patients and six HC, and TGFB1 expression was analyzed by RT-PCR.Results. TGF-β1 serum levels were significantly higher in SSc patients than in HC (p< 0.0001). Patients with increased TGF-β1 serum levels were more likely to have diffuse subset (p= 0.02), digital ulcers (p= 0.02), lung fibrosis (p< 0.0001), positive antitopoisomerase I (p= 0.03), and higher modified Rodnan score (p= 0.046). Most of our culture supernatant samples had undetectable levels of TGF-β1. No significant difference in TGFB1 expression was observed in the SSc skin compared with HC skin.Conclusion. Raised active TGF-β1 serum levels and their association with clinical manifestations in scleroderma patients suggest that this cytokine could be a marker of fibrotic and vascular involvement in SSc.

scholarly journals Correlation between Potential Risk Factors and Pulmonary Embolism in Sarcoidosis Patients Timely Treated

2021 ◽  
Vol 10 (11) ◽  
pp. 2462
Author(s):  
Barbara Ruaro ◽  
Paola Confalonieri ◽  
Mario Santagiuliana ◽  
Barbara Wade ◽  
Elisa Baratella ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Embolism ◽  
Potential Risk ◽  
Smoking Habit ◽  
Age At Diagnosis ◽  
Antiphospholipid Antibody ◽  
Increased Risk ◽  
Significant Difference ◽  
Antibody Positivity ◽  
Potential Risk Factors

Background. Some studies with inconclusive results have reported a link between sarcoidosis and an increased risk of pulmonary embolism (PE). This study aimed at assessing a possible correlation between potential risk factors and PE in sarcoidosis patients. Methods. A total of 256 sarcoidosis patients (84 males and 172 females; mean age at diagnosis 49 ± 13) were enrolled after giving written informed consent. Clinical evaluations, laboratory and radiology tests were performed to evaluate the presence of pulmonary embolism. Results. Fifteen sarcoidosis patients with PE (4 males and 11 females; mean age at diagnosis 50 ± 11), diagnosed by lung scintigraphy and 241 sarcoidosis patients without PE (80 males and 161 females; mean age at diagnosis 47 ± 13), were observed. There was a statistically significant increase of the presence of antiphospholipid antibodies in the sarcoidosis group with pulmonary embolism. There was no statistically significant difference between the two groups as to smoking habit, obesity or hereditary thrombophilia frequency (p > 0.05, respectively). Conclusions. This study demonstrates a significant correlation between the presence of antiphospholipid antibody positivity and the pulmonary embolism events in our sarcoidosis patients. Furthermore, we propose screening for these antibodies and monitoring, aimed at timely treatment.

Dr. Kendig Replies

PEDIATRICS ◽  
1975 ◽  
Vol 55 (3) ◽  
pp. 444-444
Author(s):  
Edwin L. Kendig
Keyword(s):  
Fungal Infection ◽  
Foreign Bodies ◽  
Geographic Area ◽  
Medical College ◽  
Common Diagnosis ◽  
The World

It should first be pointed out that in Nansemond County, Virginia, the incidence of presumptive sarcoidosis is 500 per 100,000 population, more than three times greater than that noted anywhere else in the world. Other nearby counties appear to be similarly affected and sarcoidosis is a common diagnosis in the geographic area served by the Medical College of Virginia Hospitals. Dr. Pickering correctly states that "sarcoidosis is difficult to define and protean in its manifestations and simulates many disease entitles such as tuberculosis, fungal infection, leprosy, syphilis, berylliosis, silicosis, tumor, and foreign bodies."

scholarly journals The contribution of epigenetics to the pathogenesis and gender dimorphism of systemic sclerosis: a comprehensive overview

2020 ◽  
Vol 12 ◽  
pp. 1759720X2091845 ◽  
Author(s):  
Bianca Saveria Fioretto ◽  
Irene Rosa ◽  
Eloisa Romano ◽  
Yukai Wang ◽  
Serena Guiducci ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
X Chromosome ◽  
Clinical Manifestations ◽  
Connective Tissue Disorder ◽  
Epigenetic Modifications ◽  
Unknown Etiology ◽  
Comprehensive Overview ◽  
Gender Dimorphism ◽  
Immune Related Genes ◽  
The Right

Systemic sclerosis (SSc) is a life-threatening connective tissue disorder of unknown etiology characterized by widespread vascular injury and dysfunction, impaired angiogenesis, immune dysregulation and progressive fibrosis of the skin and internal organs. Over the past few years, a new trend of investigations is increasingly reporting aberrant epigenetic modifications in genes related to the pathogenesis of SSc, suggesting that, besides genetics, epigenetics may play a pivotal role in disease development and clinical manifestations. Like many other autoimmune diseases, SSc presents a striking female predominance, and even if the reason for this gender imbalance has yet to be completely understood, it appears that the X chromosome, which contains many gender and immune-related genes, could play a role in such gender-biased prevalence. Besides a short summary of the genetic background of SSc, in this review we provide a comprehensive overview of the most recent insights into the epigenetic modifications which underlie the pathophysiology of SSc. A particular focus is given to genetic variations in genes located on the X chromosome as well as to the main X-linked epigenetic modifications that can influence SSc susceptibility and clinical phenotype. On the basis of the most recent advances, there is realistic hope that integrating epigenetic data with genomic, transcriptomic, proteomic and metabolomic analyses may provide in the future a better picture of their functional implications in SSc, paving the right way for a better understanding of disease pathogenesis and the development of innovative therapeutic approaches.

scholarly journals Changes in Plasma Phospholipid Metabolism Are Associated with Clinical Manifestations of Systemic Sclerosis

Diagnostics ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 2116
Author(s):  
Marija Geroldinger-Simić ◽  
Thomas Bögl ◽  
Markus Himmelsbach ◽  
Norbert Sepp ◽  
Wolfgang Buchberger
Keyword(s):  
Systemic Sclerosis ◽  
High Performance ◽  
Clinical Manifestations ◽  
Plasma Phospholipid ◽  
Phospholipid Metabolism ◽  
Internal Organs ◽  
New Biomarkers ◽  
First Time

Systemic sclerosis (SSc) is an autoimmune disease with fibrosis of the skin and/or internal organs, causing a decrease in quality of life and survival. There is no causative therapy, and the pathophysiology of the SSc remains unclear. Studies showed that lipid metabolism was relevant for autoimmune diseases, but little is known about the role of lipids in SSc. In the present study, we sought to explore the phospholipid profile of SSc by using the lipidomics approach. We also aimed to analyze lipidomics results for different clinical manifestations of SSc. Experiments were performed using high-performance liquid chromatography coupled to mass spectrometry for the lipidomic profiling of plasma samples from patients with SSc. Our study showed, for the first time, significant changes in the level of phospholipids such as plasmalogens and sphingomyelins from the plasma of SSc patients as compared to controls. Phosphatidylcholine plasmalogens species and sphingomyelins were significantly increased in SSc patients as compared to controls. Our results also demonstrated a significant association of changes in the metabolism of phospholipids (phosphatidylcholine and phosphatidylethanolamine plasmalogens species and sphingomyelins) with different clinical manifestations of SSc. Further lipidomic studies might lead to the detection of lipids as new biomarkers or therapeutic targets of SSc.

(A Clinicopathological Aspects of Resectable Pancreatic Neoplasm)

2021 ◽  
Vol 6 (15) ◽  
pp. 39-44
Author(s):  
Şeref Dokcu ◽  
Mehmet Ali Çaparlar ◽  
Salim Demirci
Keyword(s):  
Pancreatic Cancer ◽  
Survival Analysis ◽  
Survival Rates ◽  
Pancreatic Neoplasm ◽  
Population Based ◽  
Clinicopathological Features ◽  
Categorical Variables ◽  
Malignant Neoplasms ◽  
Continuous Variables ◽  
Common Diagnosis

Aim Pancreatic cancer is one of the deadliest malignant neoplasms. As with many malignant neoplasms, survival rates depend on the histopathological type of cancer, its stage, tumor size, and treatment. In this study, we aimed to classify pancreatic cancer according to clinicopathological features and histological subtypes. Material and method The data of all adult patients diagnosed and treated for pancreatic neoplasm in our clinic were collected retrospectively from the hospital's computerized database and medical files. Patients were categorized according to their clinicopathological features. Chi-square test and Fisher's exact test were used for between-group comparisons, and t-test was used for independent samples for quantitative data. Data were expressed as mean ± SD for continuous variables and numbers and percentages for categorical variables. A value of p<0.05 was considered significant. Results The mean age of the patients was 60.5 years, 70.8% were male. There were five types of tumors defined histopathologically, and the most common diagnosis was adenocarcinoma (76.9%). The most common localization of the tumor was head and neck (44.4%). Whipple surgery was performed predominantly in 69.2% of patients, and distal pancreatectomy in 29.0%. Postoperative complications were observed in more than one third (34%) of the patients. The main complications were pancreatic cyst (16.3%). In the survival analysis performed with the Kaplan-Meier test, median survival of 30.5 months, and overall survival (OS) at 1.2 and 5 years were 67.8%, 40.5%, and 16.6%, respectively. Discussion However, survival analysis results were optimistic compared to population-based studies as all patients had resectable tumors.

Diagnosis of pulmonary hypertension associated whith systemic sclerosis

2020 ◽  
Vol 5-6 (215-216) ◽  
pp. 15-23
Author(s):  
Nazym Junusbayeva ◽  
◽  
Bakytsholpan Issayeva ◽  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Clinical Symptoms ◽  
Survival Rates ◽  
Early Death ◽  
Daily Practice ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
The Right

Systemic sclerosis is the most common autoimmune disease associated with pulmonary hypertension. Pulmonary hypertension is a potential predictor of early death, and therefore, recently, interest in a comprehensive study of the diagnosis of pulmonary hypertension associated with systemic sclerosis has been increasing among researchers. Aim. Consideration of current diagnostic issues, as well as the principles of using diagnostic algorithms to verify pulmonary hypertension associated with systemic sclerosis, applied in daily clinical practice. Material and methods. In order to study the literature data, a search was carried out for information on this problem up to 10 years in depth in the Web of Science, PubMed/MEDLINE. During the search, the following terms were used individually and in combination: "systemic sclerosis", "pulmonary hypertension", "DETECT algorithm", "catheterization of the right heart chambers". The main search criteria were studies based on the study of patients with pulmonary hypertension associated with systemic sclerosis: meta-analyzes, original studies, retrospective and cohort studies. Results and discussion. The clinical symptoms of pulmonary hypertension can be nonspecific, which greatly complicates the diagnosis of the disease in the early stages. The absence of a specific clinical picture before the development of an advanced stage of pulmonary hypertension leads to late verification of the diagnosis. Key algorithms used in the daily practice of a doctor make it possible to minimize the number of undiagnosed cases of pulmonary hypertension. Conclusions. Diagnosis of PH associated with systemic sclerosis is often challenging for clinicians to practice. Early diagnosis and therefore treatment of PH are of paramount importance as they improve survival rates in patients with systemic sclerosis. Keywords: systemic sclerosis, pulmonary hypertension, screening, DETECT algorithm, right heart catheterization.

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