Systematic scleroderma is a rare chronic autoimmune disease of unknown aetiology. The aim of this study was to identify the prevalence of orofacial pathognomonic conditions in patients with systemic scleroderma using only randomised prospective studies that investigated the treatment of oral and maxillofacial changes, highlighted associations between the disease and Sjogren's syndrome, and/or analysed the effect of oral hygiene.
The literature was systematically reviewed based on Cochrane Library, EMBASE, PubMed, Scopus, and Web of Science articles published up to March 2020. The primary endpoint of this analysis was defined as an estimation of the prevalence of oral mucosal changes in different areas of the oral cavity (oral mucosa, tongue, lip, periodontal status, bones, and other regions) in patients suffering from scleroderma. Therefore, a systematic literature search (Cochrane Library, EMBASE, PubMed, Scopus, and Web of Science) was conducted and limited by the publication date (1950-03/2020) and the publication language (English). Extracted frequencies were pooled using methods for meta-analysis. In order to obtain the highest level of evidence, only prospective study reports were considered to be eligible.
After full-text screening, 14 (766 patients) out of 193 publications were eligible for the final analysis. Twelve studies produced reliable results in the final data sets. Calculation of the pooled effect estimate (random effects model) revealed a prevalence of 57.6% (95% CI: 40.8-72.9%) for the main area "lip". For the area "oral mucosa", a prevalence of 35.5% (95% CI: 15.7-62.0%) was calculated. The prevalence for "other regions" was only based on studies with salivary changes and was calculated to be 25.4% (95% CI: 14.2-41.3%).
The most pathognomonic conditions in the orofacial region in patients with systemic scleroderma affect the lips, oral mucosa, and salivary glands.