scholarly journals Systemic sclerosis and the COVID-19 pandemic: World Scleroderma Foundation preliminary advice for patient management

2020 ◽  
Vol 79 (6) ◽  
pp. 724-726 ◽  
Author(s):  
Marco Matucci-Cerinic ◽  
Cosimo Bruni ◽  
Yannick Allanore ◽  
Massimo Clementi ◽  
Lorenzo Dagna ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Severe Acute Respiratory Syndrome ◽  
Clinical Immunology ◽  
Patient Management ◽  
Severe Disease ◽  
Immunosuppressive Treatment ◽  
Disease Course ◽  
Clinical Questions ◽  
Frequent Presence

Due to the frequent presence of interstitial lung disease and widespread use of immunosuppressive treatment, systemic sclerosis (SSc) patients may be considered at risk for a more severe disease course and higher mortality when they develop Severe Acute Respiratory Syndrome - Coronavirus - 2 (SARS-CoV-2) virus infection. Therefore, with World Scleroderma Foundation endorsement, experts from different specialties including rheumatology, virology and clinical immunology gathered virtually to answer to the main practical clinical questions regarding SARS-CoV-2 infection coming from both patients and physicians. This preliminary advice is aligned with other national and international recommendations, adapted for SSc patients.

scholarly journals POS1232 COVID-19 IN PATIENTS WITH SYSTEMIC SCLEROSIS: ONE RHEUMATOLOGY CENTER EXPERIENCE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 899.2-899
Author(s):  
M. Starovoytova ◽  
O. Desinova ◽  
L. P. Ananyeva ◽  
O. Koneva ◽  
L. Garzanova ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Severe Disease ◽  
High Risk Group ◽  
Nasopharyngeal Swab ◽  
Frequent Use ◽  
Diffuse Form ◽  
Novel Coronavirus ◽  
Almost All ◽  
Dose Prednisone ◽  
Frequent Presence

Background:Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2) virus infection or COVID-19 is a serious problem for patients with systemic autoimmune diseases Given the serious complications, including acute lung injury, patients with systemic sclerosis (SSc), especially SSs associated with interstitial lung disease (ILD), may represent a high risk group for infection and the development of severe COVID-19.Objectives:We present an analysis of the COVID-19 course and outcomes in 110 SSc pts.Methods:The study included 147 patients with SSc. The information was clarified by means of telephone survey after 10 months of the pandemic (December 2020). Covid-19 was diagnosed when confirmed by positive oral /nasopharyngeal swab, in the presence of positive antibodies and/or characteristic symptoms, and data from chest computed tomography (CT). 110 pts (77%) out of 147 patients in the SSc registry, gave the necessary information. COVID-19 was diagnosed in 59 pts (53 %). 42 pts (71%) had SSc-ILD. Pts mean age was 54.96 (s.d.11, min 31, max 79), 83% women (49 women and 10 men). 38 pts (65%) had a limited form of SSc, 15 (26%) pts had diffuse form SSc, 6% had overlap (SSc-polymyositis (PM) and SSc had rheumatoid arthritis (RA) and 3% had visceral form of SSc). All patients received low-dose prednisone, and more than half of the pts received immunosuppressive therapy. Rituximab therapy was performed in 24 pts (41%).Results:Almost all patients had positive swab from the oral cavity/nasopharynx. And only in 4 (7%) pts nasopharyngeal swabs were negative, in these patients specific antibodies and characteristic CT changes were detected. Chest CT was performed in 51 (86%) pts. Novel coronavirus pneumonia developed in the vast majority of pts - in 46 (78 %) pts. CT1 (up to 25% of lung lesions) had 10 (17%) pts, CT2 (25-50%) – 21(36%) pts, CT 3 (50-75%) – 15(25%) pts. In 5 (8.5%) pts no changes were detected on CT. The course of COVID-19 was mild and moderate (20 (34%) pts and 18 (31%) pts respectively), severe course was observed in 21 (35%) pts, including fatal in 12 (20%) pts. Among the deceased pts, only 1 patient with SSc-PM had not had ILD, but 7 patients had been treated with rituximab.Conclusion:SSc SARS-CoV-2-infected patients may be at risk of severe disease and mortality due to the frequent presence of ILD and the frequent use of immunosuppressive, including biological, therapy.Disclosure of Interests:None declared

scholarly journals Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

2020 ◽  
pp. annrheumdis-2020-217455 ◽  
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Yannick Allanore ◽  
Margarida Alves ◽  
Cathrine Brunborg ◽  
Paolo Airó ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Organ Damage ◽  
Disease Course ◽  
Lung Function Decline ◽  
Mixed Effect ◽  
Progression Patterns

ObjectivesTo identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up.MethodsEligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models.Results826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms.ConclusionSSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.

Disease course and outcome of progressive interstitial lung disease in systemic sclerosis

2019 ◽  
Author(s):  
Anna-Maria Hoffmann-Vold ◽  
Yannick Allanore ◽  
Margarida Alves ◽  
Nicole Graf ◽  
Paolo Airò ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Disease Course

scholarly journals Anti-Ro52 autoantibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis

2019 ◽  
Vol 78 (7) ◽  
pp. 988-995 ◽  
Author(s):  
Sara Sabbagh ◽  
Iago Pinal-Fernandez ◽  
Takayuki Kishi ◽  
Ira N Targoff ◽  
Frederick W Miller ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Clinical Characteristics ◽  
Juvenile Dermatomyositis ◽  
Severe Disease ◽  
Trna Synthetase ◽  
Disease Course

ObjectivesAnti-Ro52 autoantibodies are associated with more severe interstitial lung disease (ILD) in adult myositis patients with antiaminoacyl transfer (t)RNA synthetase autoantibodies. However, few studies have examined anti-Ro52 autoantibodies in juvenile myositis. The purpose of this study was to define the prevalence and clinical features associated with anti-Ro52 autoantibodies in a large cohort of patients with juvenile myositis.MethodsWe screened sera from 302 patients with juvenile dermatomyositis (JDM), 25 patients with juvenile polymyositis (JPM) and 44 patients with juvenile connective tissue disease–myositis overlap (JCTM) for anti-Ro52 autoantibodies by ELISA. Clinical characteristics were compared between myositis patients with and without anti-Ro52 autoantibodies.ResultsAnti-Ro52 autoantibodies were found in 14% patients with JDM, 12% with JPM and 18% with JCTM. Anti-Ro52 autoantibodies were more frequent in patients with antiaminoacyl tRNA synthetase (64%, p<0.001) and anti-MDA5 (31%, p<0.05) autoantibodies. After controlling for the presence of myositis-specific autoantibodies, anti-Ro52 autoantibodies were associated with the presence of ILD (36% vs 4%, p<0.001). Disease course was more frequently chronic, remission was less common, and an increased number of medications was received in anti-Ro52 positive patients.ConclusionsAnti-Ro52 autoantibodies are present in 14% of patients with juvenile myositis and are strongly associated with anti-MDA5 and antiaminoacyl tRNA synthetase autoantibodies. In all patients with juvenile myositis, those with anti-Ro52 autoantibodies were more likely to have ILD. Furthermore, patients with anti-Ro52 autoantibodies have more severe disease and a poorer prognosis.

scholarly journals Determining progression of scleroderma-related interstitial lung disease

2018 ◽  
Vol 4 (1) ◽  
pp. 62-70 ◽  
Author(s):  
Elizabeth R Volkmann ◽  
Donald P Tashkin ◽  
Myung Sim ◽  
Grace Hyun Kim ◽  
Jonathan Goldin ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Disease Progression ◽  
Treatment Response ◽  
Current Approach ◽  
Disease Course ◽  
The Individual ◽  
Individual Disease ◽  
Predict Treatment Response

Interstitial lung disease occurs in the majority of patients with systemic sclerosis. Although interstitial lung disease is the number one cause of death in systemic sclerosis, interstitial lung disease progression rates vary considerably among patients with systemic sclerosis. Some patients with systemic sclerosis–associated interstitial lung disease have sub-clinical disease and may not derive benefit from immunosuppression, while others have a more aggressive interstitial lung disease phenotype. Reliable predictors of interstitial lung disease progression are lacking. The present review describes our current approach to monitoring systemic sclerosis–associated interstitial lung disease progression in clinical practice. To illustrate the marked heterogeneity that exists in interstitial lung disease progression rates in systemic sclerosis, this review presents the individual disease course of five unique patients with systemic sclerosis–associated interstitial lung disease who participated in the Scleroderma Lung Study II. These cases illustrate that treatment response rates vary in systemic sclerosis–associated interstitial lung disease and more research is needed to determine how to predict treatment response in systemic sclerosis–associated interstitial lung disease and to develop personalized treatment approaches for patients with this devastating disease.

scholarly journals Corrigendum to: Digital pitting scars are associated with a severe disease course and death in systemic sclerosis: a study from the EUSTAR cohort

Rheumatology ◽  
2022 ◽  
Author(s):  
Michael Hughes ◽  
Calvin Heal ◽  
Jörg Henes ◽  
Alexandra Balbir-Gurman ◽  
Jörg H W Distler ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Severe Disease ◽  
Disease Course

scholarly journals KL-6 But Not CCL-18 Is a Predictor of Early Progression in Systemic Sclerosis-related Interstitial Lung Disease

2018 ◽  
Vol 45 (8) ◽  
pp. 1153-1158 ◽  
Author(s):  
Gloria A. Salazar ◽  
Masataka Kuwana ◽  
Minghua Wu ◽  
Rosa M. Estrada-Y-Martin ◽  
Jun Ying ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Vital Capacity ◽  
Immunosuppressive Treatment ◽  
Rate Of Change ◽  
First Year ◽  
Outcome Study ◽  
Surrogate Outcomes ◽  
Early Progression

Objective.The 2 pneumoproteins, KL-6 and CCL-18, are promising biomarkers in systemic sclerosis (SSc)-related interstitial lung disease (ILD). Our goal was to determine their predictive significance for forced vital capacity % (FVC%) decline within the first year of followup in patients with early SSc-ILD.Methods.Early SSc patients with imaging-verified ILD enrolled in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) cohort were included. Annualized rate of change in FVC% based on the baseline and followup measurement within 12–18 months was used as the surrogate outcomes for ILD progression.Results.Eighty-two early SSc-ILD patients with mean disease duration of 2.3 years were investigated. FVC% change ranged from −23% to 38%. Baseline KL-6 levels were higher in patients than healthy controls (p < 0.0001). Higher KL-6 levels were predictive of faster FVC% decline at the 1-year followup (r = −0.23, p = 0.037). Upon categorizing KL-6 using a previously published cutoff of 1273 U/ml, its predictive significance remained in the univariable model (b = −0.07, p = 0.01), indicating that patients with positive KL-6 had on average 7% more decline in annualized percent change of FVC%. Moreover, KL-6 remained an independent predictor after adjustment for sex, disease type, anti-Scl-70, and immunosuppressive treatment status in multivariable models. Although CCL-18 was higher in patients than controls (p < 0.001), its levels did not predict FVC decline rate (p = 0.458).Conclusion.KL-6 but not CCL-18 is predictive of early SSc-ILD progression. KL-6 is a promising pneumoprotein that can contribute to SSc-ILD clinical trial enrichment.

Interstitial lung disease in systemic sclerosis quantification of disease classification and progression with high-resolution computed tomography: An observational study

2021 ◽  
pp. 239719832098537
Author(s):  
Johan Clukers ◽  
Maarten Lanclus ◽  
Dennis Belmans ◽  
Cedric Van Holsbeke ◽  
Wilfried De Backer ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Severe Disease ◽  
Quantitative Computed Tomography ◽  
High Resolution Computed Tomography ◽  
Function Tests

Introduction: Systemic sclerosis–associated interstitial lung disease accounts for up to 20% of mortality in these patients and has a highly variable prognosis. Functional respiratory imaging, a quantitative computed tomography imaging technique which allows mapping of regional information, can provide a detailed view of lung structures. It thereby shows potential to better characterize this disease. Purpose: To evaluate the use of functional respiratory imaging quantitative computed tomography in systemic sclerosis–associated interstitial lung disease staging, as well as the relationship between short-term changes in pulmonary function tests and functional respiratory imaging quantitative computed tomography with respect to disease severity. Materials and methods: An observational cohort of 35 patients with systemic sclerosis was retrospectively studied by comparing serial pulmonary function tests and in- and expiratory high-resolution computed tomography over 1.5-year interval. After classification into moderate to severe lung disease and limited lung disease (using a hybrid method integrating quantitative computed tomography and pulmonary function tests), post hoc analysis was performed using mixed-effects models and estimated marginal means in terms of functional respiratory imaging parameters. Results: At follow-up, relative mean forced vital capacity percentage change was not significantly different in the limited (6.37%; N = 13; p = 0.053) and moderate to severe disease (−3.54%; N = 16; p = 0.102) groups, respectively. Specific airway resistance decreased from baseline for both groups. (Least square mean changes −25.11% predicted ( p = 0.006) and −14.02% predicted ( p = 0.001) for limited and moderate to severe diseases.) In contrast to limited disease from baseline, specific airway radius increased in moderate to severe disease by 8.57% predicted ( p = 0.011) with decline of lower lobe volumes of 2.97% predicted ( p = 0.031). Conclusion: Functional respiratory imaging is able to differentiate moderate to severe disease versus limited disease and to detect disease progression in systemic sclerosis.

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