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

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

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

The Journal of Rheumatology ◽

10.3899/jrheum.171443 ◽

2018 ◽

Vol 45 (9) ◽

pp. 1281-1288 ◽

Cited By ~ 4

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.

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

Disease Markers ◽

10.1155/2016/6064830 ◽

2016 ◽

Vol 2016 ◽

pp. 1-6 ◽

Cited By ~ 13

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.

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Role and effectiveness of complex and supervised rehabilitation on overall and hand function in systemic sclerosis patients—one-year follow-up study

Scientific Reports ◽

10.1038/s41598-021-94549-y ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Michał Waszczykowski ◽

Bożena Dziankowska-Bartkowiak ◽

Michał Podgórski ◽

Jarosław Fabiś ◽

Arleta Waszczykowska

Keyword(s):

Systemic Sclerosis ◽

Hand Function ◽

Exercise Program ◽

Rehabilitation Program ◽

Long Term Results ◽

Home Exercise ◽

Control Group ◽

Study Group

AbstractThe aim of this study was to estimate the long-term results of complex and supervised rehabilitation of the hands in systemic sclerosis (SSc) patients. Fifty-one patients were enrolled in this study: 27 patients (study group) were treated with a 4-week complex, supervised rehabilitation protocol. The control group of 24 patients was prescribed a home exercise program alone. Both groups were evaluated at baseline and after 1-, 3-, 6-, and 12-months of follow-up with the Disability of the Arm, Shoulder and Hand Questionnaire (DAHS) as the primary outcome, pain (VAS—visual analog scale), Cochin Hand Function Scale (CHFS), Health Assessment Questionnaire Disability Index (HAQ-DI), Scleroderma-HAQ (SHAQ), range of motion (d-FTP—delta finger to palm, Kapandji finger opposition test) and hand grip and pinch as the secondary outcomes. Only the study group showed significant improvements in the DASH, VAS, CHFS and SHAQ after 1, 3 and 6 months of follow-up (P = 0.0001). Additionally, moderate correlations between the DASH, CHFS and SHAQ (R = 0.7203; R = 0.6788; P = 0.0001) were found. Complex, supervised rehabilitation improves hand and overall function in SSc patients up to 6 months after the treatment but not in the long term. The regular repetition of this rehabilitation program should be recommended every 3–6 months to maintain better hand and overall function.

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German oncology certification system for colorectal cancer – relative survival rates of a single certified centre vs. national and international registry data

Innovative Surgical Sciences ◽

10.1515/iss-2021-0002 ◽

2021 ◽

Vol 0 (0) ◽

Author(s):

Maximilian Richter ◽

Lena Sonnow ◽

Amir Mehdizadeh-Shrifi ◽

Axel Richter ◽

Rainer Koch ◽

...

Keyword(s):

Colorectal Cancer ◽

Rectal Cancer ◽

International Comparison ◽

Cancer Registry ◽

Survival Data ◽

Survival Rates ◽

Relative Survival ◽

Registry Data ◽

Cancer Registry Data ◽

Colon And Rectal Cancer

Abstract Objectives To evaluate how the certification of specialised Oncology Centres in Germany affects the relative survival of patients with colorectal cancer (CRC) by means of national and international comparison. Methods Between 2007 and 2013, 675 patients with colorectal cancer, treated at the Hildesheim Hospital, an academic teaching hospital of the Hannover Medical School (MHH), were included. A follow-up of the entire patient group was performed until 2014. To obtain international data, a SEER-database search was done. The relative survival of 148,957 patients was compared to our data after 12, 36 and 60 months. For national survival data, we compared our rates with 41,988 patients of the Munich Cancer Registry (MCR). Results Relative survival at our institution tends to be higher in advanced tumour stages compared to national and international cancer registry data. Nationally we found only little variation in survival rates for low stages CRC (UICC I and II), colon, and rectal cancer. There were notable variations regarding relative survival rates for advanced CRC tumour stages (UICC IV). These variations were even more distinct for rectal cancer after 12, 36 and 60 months (Hildesheim Hospital: 89.9, 40.3, 30.1%; Munich Cancer Registry (MCR): 65.4, 28.7, 16.6%). The international comparison of CRC showed significantly higher relative survival rates for patients with advanced tumour stages after 12 months at our institution (77 vs. 54.9% for UICC IV; raw p<0.001). Conclusions Our findings suggest that patients with advanced tumour stages of CRC and especially rectal cancer benefit most from a multidisciplinary and guidelines-oriented treatment at Certified Oncology Centres. For a better evaluation of cancer treatment and improved national and international comparison, the creation of a centralised national cancer registry is necessary.

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Sinonasal mucosal melanoma: treatment strategies and survival rates for a rare disease entity

Wiener klinische Wochenschrift ◽

10.1007/s00508-021-01847-6 ◽

2021 ◽

Author(s):

Alexandros Andrianakis ◽

Peter Kiss ◽

Markus Pomberger ◽

Axel Wolf ◽

Dietmar Thurnher ◽

...

Keyword(s):

Rare Disease ◽

Survival Data ◽

Survival Rates ◽

Delayed Diagnosis ◽

Distant Metastases ◽

Primary Treatment ◽

Mucosal Melanoma ◽

Disease Entity ◽

Improve Outcome ◽

Initial Symptoms

Summary Background Sinonasal mucosal melanoma (SNMM) is a rare disease entity comprising 0.4–1.3% of all melanomas. Surgery with free margins has been the primary treatment over decades. Neither the addition of radiotherapy nor chemotherapy could significantly improve outcome rates of this devastating malignancy. This study presents our clinical experience with SNMM over a 19-year period and summarizes the current body of literature on SNMM. Methods This retrospective analysis included 12 patients with SNMM treated from 2001 to 2019 at an academic center. Additionally, a literature review of the last 29 years on treatment and survival data of SNMM was conducted. Results Main initial symptoms were epistaxis and nasal obstruction. Of the patients 9 underwent endoscopic surgery, 6 received adjuvant therapy. 3 patients who did not undergo surgery, received chemoradiotherapy, radiotherapy alone, and chemotherapy alone, respectively. At the time of diagnosis 2 patients had distant metastases and 4 patients developed distant metastases during the course of the disease. Mean overall survival (OS) was 30.6 months, 3‑year and 5‑year OS were 25%, and 18.2%, respectively. Conclusion Unspecific symptoms and hidden anatomic locations lead to delayed diagnosis and increased rates of metastatic dissemination. Distant metastasis is the main treatment failure in SNMM. Surgery with free margins remains the primary treatment for SNMM. Adjuvant radiotherapy might improve local control in individual cases but efficient systemic therapy is needed to improve outcome rates. To evaluate and define more effective targeted treatment options and improve outcome rates, homogeneous data and prospective multicentric analysis are needed.

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EPID-10. GLIOMA INCIDENCE AND SURVIVAL BY INCOME LEVEL IN THE UNITED STATES GENERAL POPULATION AGES AND ABOVE FROM 2000-2018

Neuro-Oncology ◽

10.1093/neuonc/noab196.343 ◽

2021 ◽

Vol 23 (Supplement_6) ◽

pp. vi87-vi88

Author(s):

Jennifer Murillo ◽

Elizabeth Anyanda ◽

Jason Huang

Keyword(s):

United States ◽

General Population ◽

Survival Data ◽

Survival Rates ◽

The United States ◽

Income Level ◽

Population Based ◽

Research Database ◽

Income Levels ◽

Average Survival

Abstract Gliomas are the most common primary malignant brain tumor in the United States with previous studies showing the incidence varied by age, sex, and race or ethnicity. Survival after diagnosis has also been shown to vary by these factors. Also, socioeconomic status and its association with various cancers have also been studied at length over time. PURPOSE: The purpose of our research was to quantify the differences in incidence and survival rates of gliomas in 15 years and older by income level. METHODS: This population-based study obtained incidence and survival data from the Incidence-SEER Research Database the general population. Average age incidence were generated by glioma groups and grouped by income levels. Survival rates were generated by overall glioma diagnosis grouped by observed survival at 12, 24, 36, 48 and 60 months and by again by income levels. The analysis included 94,207 patients with glioma diagnosed in those aged 15 years or older. RESULTS: Overall, 94, 207 patients diagnosed with glioma were analyzed. Of these, 1,089 (1.16%) fell into the < $35k group, 1,684 (1.79%) in the $35k-$40k group, 3,473 (3.69%) in the $40k-$45k group, 5,647 (5.99%) in the $45k-$50k group, 7,138 (7.58%) in the $50k-$55k group, 6,468 (6.87%) in the $55k-$60k group, 15,348 (16.29%) in the $60k-$65k group, 13,216 (14.03%) in the $65k-$70k group, 9,035 (9.59%) in the $70k-$75k group, and 31,109 (33.02%) fell in > $75k group. The data was also broken further down into survivability showing average survival. CONCLUSION: Incidence of glioma and 12, 24, 36, 48 and 60 month survival rates after diagnosis vary significantly by income level with higher income level greater than $75,000+ having higher incidence and higher survival rates compared with lower income levels. Further research is needed to help determine risk factors and barriers to care to help reveal health disparities.

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Relation Between Egg Size, Growth, and Natural Mortality of Larval Fish

Journal of the Fisheries Research Board of Canada ◽

10.1139/f75-288 ◽

1975 ◽

Vol 32 (12) ◽

pp. 2503-2512 ◽

Cited By ~ 218

Author(s):

D. M. Ware

Keyword(s):

Death Rate ◽

Egg Size ◽

Larval Fish ◽

Juvenile Fish ◽

Survival Rates ◽

Fish Eggs ◽

Trophic Relations ◽

Size Growth ◽

Dependent Growth ◽

Eggs And Larvae

A set of density-dependent growth and survivorship equations is derived from evidence that the instantaneous death rate in the sea is inversely proportional to particle size. The survivorship equation reproduces several well-known phenomena observed in fish populations. It predicts: 1) that winter and spring spawning species ought to produce larger eggs than summer spawners, 2) that it is advantageous for species that spawn in batches to produce progressively smaller eggs in spring and summer, and 3) that the death rate of a cohort of fish should decrease continuously as the survivors grow and approach the critical size.The biological basis for the observed variation in the size of pelagic fish eggs and larvae is thought to be due primarily to trophic relations within the pelagic community. It is suggested from what is known of the relative abundance and foraging capabilities of different sized particles, that the survival rates of larval and juvenile fish should increase as they grow and occupy a progressively higher position in the food chain.

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

Therapeutic Advances in Musculoskeletal Disease ◽

10.1177/1759720x20918456 ◽

2020 ◽

Vol 12 ◽

pp. 1759720X2091845 ◽

Cited By ~ 1

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.

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