OBJECTIVES : Systemic sclerosis (SSc) is a rare systemic autoimmune disease characterized by a multifactorial aetiology. This severe pathology is often diagnosed late, leads to various complications, difficulties of daily functioning and social adaptation and high mortality rate. It has a significant negative impact on both physical and mental patient’s quality of life (HRQoL) and burden for patient’s caregivers. Therefore, SSc is associated with financial impact on the healthcare system. The objective of the study was to evaluate the economic burden of the SSc.

METHODS : The systematic review was conducted in accordance with PRISMA guidelines using specified PICO(S) criteria. The search was carried out in EMBASE, MEDLINE, Cochrane databases using MeSH terms («Cost of illness» OR «Economic burden» OR synonyms) AND («Systemic Sclerosis» OR synonyms) among studies published in English from December 2009 to December 2019.

RESULTS : Total of 934 studies were identified from all databases. Out of them 153 publications were selected for eligibility, of which 9 studies met the inclusion criteria. Over the last decade the annual direct costs per adult patient with SSc were found to be almost similar in different countries: 1,413-17,300 EUR (an average 8,000 EUR) in European countries, 5,038 CAD in Canada, 11,607 AUD in Australia, 17,365-22,016 USD in the USA. Hospitalization and drug therapy accounted for the greatest percentage of direct medical costs. Lung lesions and gastrointestinal problems were the most important cost drivers among the disease clinical manifestations. Costs for the diffuse cutaneous form of SSc were found to be statistically higher than for the limited form. Indirect costs are much higher compared to the general population and dominated by loss of productivity and early retirement.

CONCLUSIONS : SSc is associated with high healthcare resource use that include both direct and indirect costs. Moreover, the economic burden of this disease is increasing worldwide.