Sicca syndrome in systemic sclerosis: a narrative review on a neglected issue

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Abstract

SSc is an auto-immune disease characterized by life-threatening manifestations such as lung fibrosis or pulmonary arterial hypertension. Symptoms with a detrimental impact on quality of life are also reported and sicca syndrome (xerostomia, xeropthalmia) is present in up to 80% of patients with SSc. Sicca syndrome can occur in the absence of overlap with Sjögren’s disease and recent studies highlight that fibrosis of minor and major salivary glands, directly linked to the pathogenesis of SSc, could be a major contributor of xerostomia in SSc. This narrative review provides an overview of the clinical presentation, diagnostic strategies, management and future perspectives on sicca syndrome in patients with SSc.

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Most cited references 72

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Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis. Some manifestations of the disease, such as scleroderma renal crisis, pulmonary arterial hypertension, digital ulceration, and gastro-oesophageal reflux, are now treatable. However, the burden of non-lethal complications associated with systemic sclerosis is substantial and is likely to become more of a challenge. Here, we review the clinical features of systemic sclerosis and describe the best practice approaches for its management. Furthermore, we identify future areas for development.

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Author and article information

Contributors

Alain Lescoat: (View ORCID Profile)

Journal

Title: Rheumatology

Publisher: Oxford University Press (OUP)

ISSN (Print): 1462-0324

ISSN (Electronic): 1462-0332

Publication date Created: February 01 2023

Publication date Created: February 06 2023

Publication date Created: July 22 2022

Publication date Other: February 01 2023

Publication date (Print): February 06 2023

Publication date (Electronic): July 22 2022

Volume: 62

Issue: SI

Pages: SI1-SI11

Article

DOI: 10.1093/rheumatology/keac412

PubMed ID: 35866609

SO-VID: 0156d0a0-795d-4929-8547-bca658d1d74f

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https://academic.oup.com/pages/standard-publication-reuse-rights

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