Systemic sclerosis, silica exposure and cellular therapies: The sand in the gears?

Rev Med Interne

Unité de médecine interne (UF04): CRMR MATHEC, maladies auto-immunes et thérapie cellulaire, centre de référence des maladies auto-immunes systémiques rares d'Île-de-France, recherche clinique en hématologie, immunologie et transplantation, URP3518, hôpital St-Louis, AP-HP, université Paris Cité, IRSL, 75010 Paris, France; Department of Medicine, McGill University, H3A 1A1 Montreal, Canada.

Published: July 2024

Systemic sclerosis (SSc) is a chronic orphan autoimmune disease with the highest mortality rate among rheumatic diseases. SSc-related interstitial-lung disease (ILD) remains among the leading causes of SSc-related mortality with still few therapeutic effective strategies. In patients with crystallin silica exposure, SSc is recognized as an occupational disease according to the French social security system (Table 25A of the general insurance regimen). Lympho-ablative or myeloablative immunosuppression followed by autologous hematopoietic stem-cell transplantation (aHSCT) is the only therapeutic approach with demonstrated efficacy, improved survival with disease modifying effects on SSc-fibrotic manifestations (skin disease and ILD) and quality of life. A documented past and/or present occupational silica exposure, with extensive exposure and/or silica-related ILD and/or with persistent silica content in the broncho-alveolar lavage fluid are contra-indications to aHSCT in SSc patients, due to the risk of silica-related malignancy or of SSc relapse. This article aims to discuss alternative options in SSc patients with a history of silica exposure, and how innovative cellular therapies (mesenchymal stromal cells, CAR cells) could represent new therapeutic options for these patients.

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http://dx.doi.org/10.1016/j.revmed.2024.02.003DOI Listing

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