The burden of illness of the organ manifestations of systemic sclerosis: a pragmatic, targeted review.

Objectives: This structured, targeted literature review aimed to assess the mortality, humanistic and economic burden of eight organ manifestations which are commonly experienced by systemic sclerosis patients.

Methods: Identification of relevant literature was carried out by searching in Ovid MEDLINE and EMBASE, PubMed, and NHS Economic Evaluation Database in August 2023. Studies reporting original data on patients with systemic sclerosis with at least one of eight organ manifestations (interstitial lung disease and/or pulmonary hypertension, skin, peripheral vascular, musculoskeletal, gastrointestinal, cardiac or renal involvement) published within the last 15 years were included. Meta-analyses with no publication limits were also included.

Results: A total of 50 studies were identified; 37 reported mortality outcomes (including 4 meta-analyses), 9 reported humanistic burden and 11 reported economic burden outcomes. Pulmonary hypertension, cardiac and renal manifestations were generally associated with a poorer survival prognosis. Furthermore, gastrointestinal, skin and peripheral vascular manifestations were found to negatively impact health-related quality of life outcomes. Pulmonary manifestations were associated with substantial economic costs; however, the cost burden of other manifestations is insufficiently reported, despite evidence that they often require healthcare resource use.

Conclusions: Organ manifestations experienced by patients with systemic sclerosis significantly affect patient quality of life and mortality. The economic burden of organ manifestations that are widely experienced by SSc patients such as gastrointestinal issues, is poorly understood and requires further research to quantify and understand. Improvements in diagnosis and clinical management of these systemic sclerosis-associated organ manifestations have the potential for significant alleviation of disease-related burdens.