Defining primary systemic sclerosis heart involvement: A scoping literature review

Area of Science:

• Rheumatology
• Cardiology
• Systemic Autoimmune Diseases

Background:

• Clinically evident primary heart involvement in systemic sclerosis (SHI) is a poor prognostic factor and a leading cause of death in systemic sclerosis (SSc).
• Currently, there is no consensus definition for SHI, leading to a poor understanding of its natural history and risk factors.

Purpose of the Study:

• To conduct a scoping literature review to identify definitions and measurement tools for SHI.
• To capture factors associated with an increased risk of developing SHI.

Main Methods:

• A scoping literature review was performed on articles focusing on SHI.
• Searches were conducted in MEDLINE, EMBASE, and PubMed databases.
• 295 records were included in the final review from an initial 2436 identified.

Main Results:

• The review identified studies of variable quality with generally low patient numbers.
• Highly heterogeneous definitions of SHI were found across studies.
• There is no clear consensus on the scope of SHI or the prognostic significance of sub-clinical abnormalities.

Conclusions:

• The lack of a standardized definition for SHI is a significant unmet need in SSc research.
• This review will aid in developing consensus classification criteria for SHI.
• The findings will facilitate accurate quantification of SHI burden, identification of risk factors, and evaluation of therapeutic strategies.