Systemic sclerosis: Update for oral health care providers

Abstract

Systemic sclerosis (SSc), also known as scleroderma, is an autoimmune disease of unknown origin characterized by an uncontrolled inflammatory process resulting in fibrosis of the skin, internal organs and vasculopathy. Manifestations of SSc are heterogenous and can include pulmonary, cardiac, neural, renal, muscular, cutaneous and orofacial complications. Recent scientific advances have led to a better understanding of disease etiopathogenesis and the development of a new classification system. Therapeutic management is often multidisciplinary and targeted toward the affected organs. Oral health care providers (OHCPs) should be familiar with SSc, particularly as it relates to its impact on the orofacial region and modifications to delivery of oral health care for patients with this condition.

Investigation of the Expression of Inflammatory Markers in Oral Biofilm Samples in Patients with Systemic Scleroderma and the Association with Clinical Periodontal Parameters—A Preliminary Study

“…Basically, it is known that TNF-α, IL-6 or IL-1 are released and attract further B-and T-cell controlled cells, thereby initiating destruction [14]. Although the exact mechanism of the development of SSc remains unclear, it is known that it also develops based on an immune response and affects the vascular system, internal organs and skin with excessive fibrosis and degradation of connective tissue [1,3]. Due to fibrosis of the skin, many orofacial changes also result, especially restricted mouth opening [16].…”

“…Coupled with limited movement of the fingers and hands and sclerodactyly, patients with SSc suffer from limited oral hygiene ability [26]. Studies have also observed decreased QoL in this context [1,16]. In addition, a recent study showed that patients with dcSSc (n = 28) in particular had a decreased salivary flow rate with reduced pH values, which correlated positively with DMFT and mRSS [27].…”

“…Systemic scleroderma (SSc) is a complex chronic inflammatory autoimmune multisystem disease characterized by vasculopathy and fibrosis of the skin and internal organs [1]. It has been reported that 1 in 10,000 people are affected by SSc worldwide, with a prevalence between 38 and 341 total cases/million individuals [2,3].…”

“…The frequency peaks are between 45 and 64 years of age and three times more frequent in females [4]. From the time of diagnosis, the cumulative survival rate is 75% at 5 years, with SSc continuing to have the highest mortality compared to other rheumatic diseases, with heart failure or lethal cardiac arrythmias being the most common causes of death [1,5,6]. The clinical manifestations of SSc mainly include skin tightness, puffy fingers, sclerodactyly and digital open ulcers [1].…”

“…From the time of diagnosis, the cumulative survival rate is 75% at 5 years, with SSc continuing to have the highest mortality compared to other rheumatic diseases, with heart failure or lethal cardiac arrythmias being the most common causes of death [1,5,6]. The clinical manifestations of SSc mainly include skin tightness, puffy fingers, sclerodactyly and digital open ulcers [1]. The extension and degree of SSc is evaluated using the modified Rodnan Skin Score (mRSS), and the definitive diagnosis will require the fulfilment of the criteria according to the 2013 European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) [7].…”