Predictors of progression in systemic sclerosis patients with interstitial lung disease
Oliver Distler,Shervin Assassi,Vincent Cottin,Maurizio Cutolo,Sonye K. Danoff,Christopher P. Denton,Jörg H W Distler,Anna Maria Hoffmann-Vold,Sindhu R. Johnson,Ulf Müller Ladner,Vanessa Smith,Vanessa Smith,Elizabeth R. Volkmann,Toby M. Maher +13 more
TLDR
Methods used to define and predict disease progression in SSc-ILD are reviewed and a strategy that combines both lung function tests and chest imaging is recommended, in the absence of standardised methods for doctors, is recommended.Abstract:
Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc. There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients9 clinical courses are variable and difficult to predict, although certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, although there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD. There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5–9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1–2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.read more
Citations
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Journal ArticleDOI
Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database.
Anna-Maria Hoffmann-Vold,Yannick Allanore,Margarida Alves,Cathrine Brunborg,Paolo Airò,L. Ananieva,László Czirják,Serena Guiducci,Eric Hachulla,Mengtao Li,C. Mihai,Gabriela Riemekasten,Petros P. Sfikakis,Otylia Kowal-Bielecka,Antonella Riccardi,Oliver Distler +15 more
TL;DR: SSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important, and novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.
Journal ArticleDOI
Guidelines for biomarkers in autoimmune rheumatic diseases - evidence based analysis.
Roberto Giacomelli,Antonella Afeltra,Alessia Alunno,E Bartoloni-Bocci,Onorina Berardicurti,Michele Bombardieri,Alessandra Bortoluzzi,Roberto Caporali,Francesco Caso,Ricard Cervera,Maria Sole Chimenti,Paola Cipriani,Emmanuel Coloma,Fabrizio Conti,Salvatore D'Angelo,Salvatore De Vita,Salvatore Di Bartolomeo,Oliver Distler,Andrea Doria,Eugen Feist,Benjamin A Fisher,Maria Gerosa,Michele Gilio,Giuliana Guggino,Vasiliki Liakouli,Domenico Paolo Emanuele Margiotta,Pier Luigi Meroni,Gianluca Moroncini,Federico Perosa,Marcella Prete,Roberta Priori,Chiara Rebuffi,Piero Ruscitti,Raffaele Scarpa,Yehuda Shoenfeld,Monica Todoerti,Francesco Ursini,Guido Valesini,Serena Vettori,Claudio Vitali,Athanasios G. Tzioufas +40 more
TL;DR: The overarching aim of this work was to clarify the meaning of specific biomarkers during autoimmune diseases; their possible role in confirming diagnosis, predicting outcome and suggesting specific treatments.
Journal ArticleDOI
68Ga-FAPI-04 PET-CT for molecular assessment of fibroblast activation and risk evaluation in systemic sclerosis-associated interstitial lung disease: a single-centre, pilot study
Christina Bergmann,Jörg H W Distler,Christoph Treutlein,Koray Tascilar,Anna-Theresa Müller,Armin Atzinger,Alexandru-Emil Matei,Johannes Knitza,Andrea-Hermina Györfi,Anja Lück,Clara Dees,Alina Soare,Andreas Ramming,Verena Schönau,Oliver Distler,Olaf Prante,Philipp Ritt,Theresa Ida Götz,Markus Köhner,Michael Cordes,Tobias Bäuerle,Torsten Kuwert,Georg Schett,Christian Schmidkonz +23 more
TL;DR: This study presents the first in-human evidence that fibroblast activation correlates with fibrotic activity and disease progression in the lungs of patients with systemic sclerosis-associated ILD and that 68Ga-FAPI-04 PET-CT might improve risk assessment of systemic MS.
Journal ArticleDOI
Systemic Sclerosis-Associated Interstitial Lung Disease: How to Incorporate Two Food and Drug Administration-Approved Therapies in Clinical Practice.
Dinesh Khanna,Alain Lescoat,Alain Lescoat,David Roofeh,Elana J. Bernstein,Ella A. Kazerooni,Michael D. Roth,Fernando J. Martinez,Kevin R. Flaherty,Christopher P. Denton +9 more
TL;DR: In this article, the authors present a practical classification of systemic sclerosis patients in terms of disease severity and associated risk of progression (low vs. high risk) and propose a practical approach for diagnosis, stratification, management, and therapeutic decision-making in this clinical context.
Journal ArticleDOI
Interstitial lung diseases
TL;DR: In this article , the authors provide an update on epidemiology, pathogenesis, presentation, diagnosis, disease course, and management of interstitial lung diseases that are most frequently encountered in clinical practice.
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