Background: 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. Methods: 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. Results: 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%). Conclusion: The most pathognomonic conditions in the orofacial region in patients with systemic scleroderma affect the lips, oral mucosa, and salivary glands.

中文翻译：

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系统性硬皮病患者口腔颌面部疾病患病率的系统评价

背景：系统性硬皮病是一种罕见的病因不明的慢性自身免疫性疾病。这项研究的目的是仅使用随机前瞻性研究来鉴定系统性硬皮病患者的口腔病理性疾病状况，这些研究调查了口腔和颌面部变化的治疗，突出了疾病与干燥综合征之间的关联性，和/或分析了口腔卫生。方法：根据Cochrane图书馆，EMBASE，PubMed，Scopus和Web of Science截至2020年3月的文章对文献进行系统地回顾。该分析的主要终点是对不同地区口腔粘膜变化发生率的估计口腔（口腔粘膜，舌头，嘴唇，牙周状况，骨骼，和其他地区）患有硬皮病的患者。因此，进行了系统的文献检索（Cochrane图书馆，EMBASE，PubMed，Scopus和Web of Science），并且受其出版日期（1950-03 / 2020）和出版语言（英语）的限制。使用荟萃分析将提取的频率合并。为了获得最高水平的证据，只有前瞻性研究报告才被认为是合格的。结果：全文筛选后，有193篇出版物中的14篇（766例患者）符合最终分析的条件。十二项研究在最终数据集中产生了可靠的结果。合并效应估计值（随机效应模型）的计算显示，主要区域“嘴唇”的患病率为57.6％（95％CI：40.8-72.9％）。对于“口腔粘膜”区域，计算患病率为35.5％（95％CI：15.7-62.0％）。“其他地区”的患病率仅基于唾液变化的研究，经计算为25.4％（95％CI：14.2–41.3％）。结论：系统性硬皮病患者的口面部区域中的大多数病理因素会影响嘴唇，口腔粘膜和唾液腺。