Ongoing Clinical Trials and Treatment Options for Patients with Systemic Sclerosis-associated Interstitial Lung Disease

Overview

Journal Rheumatology (Oxford)

Publisher Oxford University Press

Specialty Rheumatology

Date 2018 Jun 13

PMID 29893938

Citations 28

Authors

Dinesh Khanna

Donald P Tashkin

Christopher P Denton

Martin W Lubell

Cristina Vazquez-Mateo

Stephen Wax

Affiliations

Soon will be listed here.

Abstract

SSc is a rare CTD that affects multiple organ systems, resulting in substantial morbidity and mortality. Evidence of interstitial lung disease (ILD) is seen in ∼80% of patients with SSc. Currently there is no approved disease-modifying treatment for ILD and few effective treatment options are available. CYC is included in treatment guidelines, but it has limited efficacy and is associated with toxicity. MMF is becoming the most commonly used medication in clinical practice in North America and the UK, but its use is not universal. Newer agents targeting the pathogenic mechanisms underlying SSc-ILD, including fibrotic and inflammatory pathways, lymphocytes, cell-cell and cell-extracellular membrane interactions, hold promise for better treatment outcomes, including improved lung function, patient-related outcomes and quality of life. Here we review ongoing trials of established and novel agents that are currently recruiting patients with SSc-ILD.

Citing Articles

Mitochondrial DNA Sensing Pathogen Recognition Receptors in Systemic Sclerosis Associated Interstitial Lung Disease: A Review.

Ghincea A, Woo S, Yu S, Pivarnik T, Fiorini V, Herzog E Curr Treatm Opt Rheumatol. 2024; 9(4):204-220.

PMID: 38230363 PMC: 10791121. DOI: 10.1007/s40674-023-00211-1.

Diagnosis of interstitial lung disease (ILD) secondary to systemic sclerosis (SSc) and rheumatoid arthritis (RA) and identification of 'progressive pulmonary fibrosis' using chest CT: a narrative review.

Poerio A, Carlicchi E, Zompatori M Clin Exp Med. 2023; 23(8):4721-4728.

PMID: 37803100 DOI: 10.1007/s10238-023-01202-1.

Experience With Nintedanib in Severe Pulmonary Fibrosis Associated With Systemic Sclerosis: A Case Series.

Bordas-Martinez J, Llanos-Gonzalez A, Jodar-Masanes R, Vicens-Zygmunt V, Bermudo G, Luburich P Open Respir Arch. 2023; 3(1):100080.

PMID: 37497357 PMC: 10369530. DOI: 10.1016/j.opresp.2020.100080.

Clinical phenotype in scleroderma patients based on autoantibodies.

Sieiro Santos C, Moriano Morales C, Alvarez Castro C, Alvarez E Rheumatol Adv Pract. 2023; 7(Suppl 1):i26-i33.

PMID: 36968636 PMC: 10036993. DOI: 10.1093/rap/rkad010.

Pharmacological treatment for connective tissue disease-associated interstitial lung involvement: Protocol for an overview of systematic reviews and meta-analyses.

Karassa F, Bougioukas K, Pelechas E, Skalkou A, Argyriou E, Haidich A PLoS One. 2022; 17(8):e0272327.

PMID: 35921316 PMC: 9348721. DOI: 10.1371/journal.pone.0272327.

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