Expert Consensus on the Screening, Treatment, and Management of Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD), and the Potential Future Role of Anti-fibrotics[asterisk] in a Treatment Paradigm for SSc-ILD: A Delphi Consensus Study

Objective.To provide an overview of the role of lung ultrasound (LUS) in the assessment of interstitial lung disease (ILD) in systemic sclerosis (SSc) and to discuss the state of validation supporting its clinical relevance and application in daily clinical practice.Methods.Original articles published between January 1997 and October 2017 were included. To identify all available studies, a detailed search pertaining to the topic of review was conducted according to guidelines of the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA). A systematic search was performed in PubMed and EMBASE. The quality assessment of retrieved articles was performed according to the Oxford Center for Evidence-based Medicine. The methodological quality of the studies was assessed using the Cochrane Handbook for Systematic Reviews and the Quality Assessment of Diagnostic Accuracy Studies–2 tool.Results.From 300 papers identified, 12 were included for the analysis. LUS passed the filter of face, content validity, and feasibility. However, there is insufficient evidence to support criterion validity, reliability, and sensitivity to change.Conclusion.Despite a great deal of work supporting the potential role of LUS for the assessment of ILD-SSc, much remains to be done before validating its use as an outcome measure in ILD-SSc.

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POS1228 THE ROLE OF CHEST CT IN UNDERSTANDING INTERSTITIAL LUNG DISEASE (ILD): SYSTEMIC SCLEROSIS (SSc). VERSUS COVID-19

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.2967 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 897.2-898

Author(s):

M. Orlandi ◽

N. Landini ◽

G. Sambataro ◽

C. Nardi ◽

C. Bruni ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Lower Lobe ◽

Chest Ct ◽

Predictive Score ◽

Risk Class ◽

Radiological Society ◽

Radiological Score

Background:COVID-19 pandemic is a global emergency which may overlap on the clinical and radiological scenario of ILD in SSc. In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 superinfection from a progression of SSc-ILD.Objectives:The aim of our study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia.Methods:22 international readers were included and divided in the radiologist group (RAD) and non-radiologist group (nRAD). The RAD group included non-chest RAD and chest-RAD. A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study.Results:Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT parameters most frequently associated with SSc-ILD. The CT parameters most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p <0.0001) and signs of fibrosis in GGO in the lower lobes (p <0.0001) remained independently associated with COVID-19 pneumonia or SSc-ILD, respectively. These two variables were combined in a predictive score which resulted positively associated with the COVID-19 diagnosis, with 96.1% sensitivity and 83.3% specificity: 3 different risk class for COVID-19 pneumonia may be identified: high risk for COVID-19 pneumonia (5-9 points); probable overlap COVID-19 pneumonia in SSc-ILD (4 points); low risk for COVID-19 pneumonia (0-3 points).Conclusion:The CT differential diagnosis between COVID-19 Pneumonia and SSc-ILD is possible and may be fostered in practice by the use of a radiological score. In the case where an overlap of both diseases is suspected, the presence of consolidation in the lower lobes may suggest a COVID-19 pneumonia while the presence of fibrosis inside GGO may indicate a SSc-ILD.References:[1]Orlandi M, Landini N, Bruni C, et al. Infection or autoimmunity? The clinical challenge of interstitial lung disease in systemic sclerosis during COVID 19 pandemic. J Rheumatol. 2020 Dec 1: jrheum.200832[2]Simpson S, Kay FU, Abbara S, et al. Radiological Society of North America Expert Consensus Statement on Reporting Chest CT Findings Related to COVID-19. Endorsed by the Society of Thoracic Radiology, the American College of Radiology, and RSNA [published online ahead of print, 2020 Apr 28]. J Thorac Imaging. 2020;10.1097/RTI.0000000000000524.[3]Cheng C, Li C, Zhao T, et al. COVID-19 with rheumatic diseases: a report of 5 cases. Clin Rheumatol. 2020;39(7):2025-2029.[4]Mariano RZ, Rio APTD, Reis F. Covid-19 overlapping with systemic sclerosis. Rev Soc Bras Med Trop. 2020 Sep 21;53:e20200450.Disclosure of Interests:None declared

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The role of vasodilators in patients with progressive systemic sclerosis. Interstitial lung disease and pulmonary hypertension

CHEST Journal ◽

10.1378/chest.88.4.263s ◽

1985 ◽

Vol 88 (4) ◽

pp. 263S-265 ◽

Cited By ~ 1

Author(s):

J. Ohar ◽

C. Polatty ◽

A. Robichaud ◽

A. Fowler ◽

G. Vetrovec ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Progressive Systemic Sclerosis

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Economic Burden and Management of Systemic Sclerosis-Associated Interstitial Lung Disease in 8 European Countries: The BUILDup Delphi Consensus Study

Advances in Therapy ◽

10.1007/s12325-020-01541-5 ◽

2020 ◽

Cited By ~ 1

Author(s):

Jesper Rømhild Davidsen ◽

Jelle Miedema ◽

Wim Wuyts ◽

Maritta Kilpeläinen ◽

Spyridon Papiris ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Economic Burden ◽

European Countries ◽

Delphi Consensus

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AB0094 INCREASE OF ENDOTHELIAL PROGENITOR CELLS IN SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.2350 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1076.1-1076

Author(s):

V. Pulito-Cueto ◽

S. Remuzgo Martinez ◽

F. Genre ◽

B. Atienza-Mateo ◽

V. M. Mora-Cuesta ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Progenitor Cells ◽

Endothelial Progenitor Cells ◽

Potential Role ◽

Type I ◽

Research Support ◽

Endothelial Progenitor

Background:Endothelial progenitor cells (EPC), involved in vasculogenesis and endothelial tissue repair, have been described as relevant players in vascular and connective tissue diseases [1-2]. In this regard, a previous study of our group disclosed that the degree of EPC frequency may help to identify the presence of interstitial lung disease (ILD) in rheumatoid arthritis patients [3]. Given that ILD is the main cause of mortality in patients with systemic sclerosis (SSc) [1, 4-6], the understanding of the role of EPC in the mechanism of SSc-ILD+ vasculopathy is crucial.Objectives:To assess the potential role of EPC on vascular dysfunction associated with the presence of ILD in patients with SSc.Methods:Peripheral venous blood was collected from a total of 39 patients with SSc, 20 with ILD (SSc-ILD+) and 19 without ILD (SSc-ILD-). All subjects were recruited from the Rheumatology and Pneumology departments of Hospital Universitario Marqués de Valdecilla, Santander, Spain. Quantification of EPC was analyzed by flow cytometry. EPC were considered as CD34+, CD45Low, CD309+ and CD133+.Results:Statistically significant differences in EPC frequency between patients with SSc-ILD+ and patients with SSc-ILD- were disclosed. Specifically, an increase of EPC frequency was observed in SSc-ILD+ patients when compared to patients with SSc-ILD- (mean ± standard deviation: 0.033 ± 0.012 versus 0.021 ± 0.017, respectively, p=0.012).Conclusion:Our results suggest a potential role of EPC on vascular damage associated with the manifestation of ILD in patients with SSc.References:[1]Eur J Rheumatol 2020;7(Suppl 3):S139-S146.[2]Arthritis Rheum 2009;60(11):3168-79.[3]J Clin Med 2020;9(12):4098.[4]Ann Rheum Dis 2007;66(7):940-4.[5]Rheumatology (Oxford) 2010;49(12):2375-80.[6]Eur Respir Rev 2015;24(135):102-14.Acknowledgements:Personal funds, VP-C: PREVAL18/01 (IDIVAL); SR-M: RD16/0012/0009 (ISCIII-ERDF); LL-G: INNVAL20/06 (IDIVAL); RP-F: START PROJECT (FOREUM); RL-M: Miguel Servet type I CP16/00033 (ISCIII-ESF).Disclosure of Interests:Verónica Pulito-Cueto: None declared, Sara Remuzgo Martinez: None declared, Fernanda Genre: None declared, Belén Atienza-Mateo: None declared, Victor Manuel Mora-Cuesta: None declared, David Iturbe-Fernández: None declared, Leticia Lera-Gómez: None declared, Raquel Pérez-Fernández: None declared, Diana Prieto-Peña: None declared, Virginia Portilla: None declared, Ricardo Blanco Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Grant/research support from: Abbvie, MSD and Roche, Alfonso Corrales: None declared, Jose Manuel Cifrián-Martínez: None declared, Raquel López-Mejías: None declared, Miguel A González-Gay Speakers bureau: Pfizer, Abbvie, MSD, Grant/research support from: Pfizer, Abbvie, MSD

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Subclinical interstitial lung disease in patients with systemic sclerosis. A pilot study on the role of ultrasound

Reumatología Clínica (English Edition) ◽

10.1016/j.reumae.2019.05.004 ◽

2021 ◽

Vol 17 (3) ◽

pp. 144-149

Author(s):

Samuel Reyes-Long ◽

Marwin Gutierrez ◽

Denise Clavijo-Cornejo ◽

Alfonso Alfaro-Rodríguez ◽

Karen González-Sámano ◽

...

Keyword(s):

Systemic Sclerosis ◽

Pilot Study ◽

Interstitial Lung Disease ◽

Lung Disease

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Systemic sclerosis-associated interstitial lung disease: Role of the oesophagus in outcomes

Respirology ◽

10.1111/resp.13335 ◽

2018 ◽

Vol 23 (10) ◽

pp. 885-886 ◽

Cited By ~ 2

Author(s):

Mary E. Strek

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease

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Role of autoantibodies in the diagnosis and prognosis of interstitial lung disease in autoimmune rheumatic disorders

Therapeutic Advances in Musculoskeletal Disease ◽

10.1177/1759720x211032457 ◽

2021 ◽

Vol 13 ◽

pp. 1759720X2110324

Author(s):

Masataka Kuwana ◽

Albert Gil-Vila ◽

Albert Selva-O’Callaghan

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Topoisomerase I ◽

Initiation Factor ◽

Severe Condition ◽

Diagnosis And Prognosis ◽

Antisynthetase Antibodies ◽

Substantial Morbidity

Interstitial lung disease (ILD) has been recognized as a frequent manifestation associated with a substantial morbidity and mortality burden in patients with autoimmune rheumatic disorders. Serum autoantibodies are considered good biomarkers for identifying several subsets or specific phenotypes of ILD involvement in these patients. This review features the role of several autoantibodies as a diagnostic and prognostic biomarker linked to the presence ILD and specific ILD phenotypes in autoimmune rheumatic disorders. The case of the diverse antisynthetase antibodies in the antisynthease syndrome or the anti-melanoma differentiation-associated 5 protein (MDA5) antibodies as a marker of a severe condition such as rapidly progressive ILD in patients with clinically amyopathic dermatomyositis are some of the associations herein reported in the group of myositis spectrum disorders. Specific autoantibodies such as the well-known anti-topoisomerase I (anti-Scl70) or the anti-Th/To, anti-U11/U12 ribonucleoprotein, and anti-eukaryotic initiation factor 2B (eIF2B) antibodies seems to be specifically linked to ILD in patients with systemic sclerosis. Overlap syndromes between systemic sclerosis and myositis, also have good ILD biomarkers, which are the anti-PM/Scl and anti-Ku autoantibodies. Lastly, other not so often reported disorders as being associated with ILD but recently most recognized as is the case of rheumatoid arthritis associated ILD or entities herein included in the miscellaneous disorders section, which include anti-neutrophil cytoplasmic antibody-associated interstitial lung disease, Sjögren’s syndrome or the mixed connective tissue disease, are also discussed.

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