Orofacial Complications of the Connective Tissue Disease Systemic Sclerosis.

Journal of dental research Pub Date : 2024-07-01 Epub Date: 2024-05-23 DOI:10.1177/00220345241249408
M Sharma, A Fadl, A Leask
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Abstract

Scleroderma (systemic sclerosis, SSc) is an autoimmune fibrosing connective tissue disease of unknown etiology. SSc patients show increased levels of autoantibodies, profibrotic cytokines, and extracellular matrix remodeling enzymes that collectively cause activated (myo)fibroblasts, the effector cell type of fibrosis. Despite these impacts, no disease-modifying therapy exists; individual symptoms are treated on a patient-to-patient basis. SSc research has been principally focused on symptoms observed in the lung and skin. However, SSc patients display significant oral complications that arise due to fibrosis of the not only skin, causing microstomia, but also the gastrointestinal tract, causing acid reflux, and the oral cavity itself, causing xerostomia and gingival recession. Due to these complications, SSc patients have impaired quality of life, including periodontitis, tooth loss, reduced tongue mobility, and malnutrition. Indeed, due to their characteristic oral presentation, SSc patients are often initially diagnosed by dentists. Despite their clinical importance, the oral complications of SSc are severely understudied; high-quality publications on this topic are scant. However, SSc patients with periodontal complications possess increased levels of matrix metalloproteinase-9 and chemokines, such as interleukin-6 and chemokine (C-X-C motif) ligand-4. Although many unsuccessful clinical trials, mainly exploring the antifibrotic effects of anti-inflammatory agents, have been conducted in SSc, none have used oral symptoms, which may be more amenable to anti-inflammatory drugs, as clinical end points. This review summarizes the current state of knowledge regarding oral complications in SSc with the goal of inspiring future research in this extremely important and underinvestigated area.

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结缔组织病系统性硬化症的口面部并发症。
硬皮病(系统性硬化症,SSc)是一种病因不明的自身免疫性纤维结缔组织病。SSc 患者体内的自身抗体、促纤维化细胞因子和细胞外基质重塑酶水平升高,共同导致纤维化的效应细胞类型--活化(肌)成纤维细胞。尽管存在这些影响,但目前尚无改变病情的疗法;只能根据患者的不同症状进行治疗。对 SSc 的研究主要集中在肺部和皮肤的症状上。然而,SSc 患者会出现严重的口腔并发症,这不仅是因为皮肤纤维化导致小口畸形,还因为胃肠道纤维化导致胃酸倒流,以及口腔本身纤维化导致口腔干燥和牙龈萎缩。由于这些并发症,SSc 患者的生活质量受到影响,包括牙周炎、牙齿脱落、舌头活动能力下降和营养不良。事实上,由于其特征性的口腔表现,SSc 患者通常由牙医进行初步诊断。尽管 SSc 具有重要的临床意义,但对其口腔并发症的研究却严重不足;有关这一主题的高质量出版物非常少。不过,有牙周并发症的 SSc 患者体内基质金属蛋白酶-9 和白细胞介素-6、趋化因子(C-X-C motif)配体-4 等趋化因子的水平都有所升高。虽然针对 SSc 进行了许多临床试验,主要是探索抗炎药物的抗纤维化作用,但这些试验都没有将口腔症状作为临床终点,而口腔症状可能更适合使用抗炎药物。本综述总结了有关 SSc 口腔并发症的知识现状,旨在激励未来在这一极其重要且研究不足的领域开展研究。
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