Received: 28 April 2020 Revised: 5 June 2020 Accepted: 9 June 2020 DOI: 10.1111/scd.12492 REVIEW Systemic sclerosis: Update for oral health care providers Fatmah J. Alhendi 1 Victoria P. Werth 2 Thomas P. Sollecito 1 Eric T. Stoopler 1 1 Department of Oral Medicine, Penn Dental Medicine, Philadelphia, Pennsylvania 2 Department of Dermatology, Philadelphia V.A. Hospital, Hospital of the University of Pennsylvania and the Veteran’s Administration Medical Center, Philadelphia, Pennsylvania Correspondence Eric T. Stoopler, DMD, FDSRCS, FDSR- CPS, University of Pennsylvania School of Dental Medicine, University in Philadel- phia, 240 South 40th Street, Room 206, Philadelphia, PA 19104. Email: ets@upenn.edu Abstract Systemic sclerosis (SSc), also known as scleroderma, is an autoimmune disease of unknown origin characterized by an uncontrolled inflammatory process result- ing 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. KEYWORDS dental treatment, physical disability, rheumatology 1 INTRODUCTION Systemic sclerosis (SSc), also called scleroderma, is an immune-mediated, complex, multisystem disease charac- terized by fibrosis of the skin, internal organs and vascu- lopathy. In recent years, the nomenclature of this disease has been replaced by more accurate terminology describ- ing a heterogenous group of diseases that are defined by clinical features, serology, and prognosis. 1 Although recent advances have resulted in improved survival in some types of SSc, it continues to have the highest mortality com- pared to any other rheumatic disease. 2,3 SSc is associ- ated with significant morbidity with variable outcomes. 4 A systematic review to assess the oral-health-related qual- ity of life (OHRQoL) of adult patients with rheumatic dis- eases concluded that rheumatic diseases with oral involve- ment, such as SSc, has lower OHRQoL due to oral and psychosocial impairments compared to other rheumatic diseases. 5 A web-based survey by Leader et al (2014) sug- gested approximately 50% of dentists (n = 269) felt they might cause harm to SSc patients because of their insuf- © 2020 Special Care Dentistry Association and Wiley Periodicals, Inc. ficient knowledge of the disease. 6 In this review, we aim to update oral health care providers (OHCPs) regarding SSc and considerations for provision of safe and effective oral health care for this patient population. 2 EPIDEMIOLOGY Epidemiological studies of SSc are inconsistent due to prior variable classification criteria and rarity of the disease. 7 It is, however, estimated that approximately 1 in 10 000 people are affected by SSc worldwide with reported prevalence between 38 and 341 total cases/million. 7,8 Inci- dence and prevalence are highest in Europe and North America and lowest in Asiatic areas, suggesting the role of population and environmental factors. 8,9 SSc frequency peaks between 45-64 years of age 10 and is approximately 8-9 times more frequent in females. 11,12 Systematic reviews of cohort studies and meta-analysis showed a cumulative survival rate of 75% at 5 years and 62.5% at 10 years in SSc from the time of diagnosis 13,14 with severe heart failure or Spec Care Dentist. 2020;1–13. wileyonlinelibrary.com/journal/scd 1