Towards developing criteria for scleroderma renal crisis: A scoping review

Area of Science:

• Rheumatology
• Nephrology
• Internal Medicine

Background:

• Scleroderma renal crisis (SRC) is a serious complication of scleroderma.
• The absence of a universally accepted gold standard definition for SRC impedes research and clinical understanding.
• Standardized criteria are crucial for accurate diagnosis and effective management of SRC.

Purpose of the Study:

• To conduct a scoping review to identify all published and unpublished definitions of SRC.
• To analyze the heterogeneity in diagnostic criteria used for SRC.
• To inform the development of a consensus definition for SRC.

Main Methods:

• A comprehensive literature search was performed across Medline, Embase, and non-Ovid Pubmed databases.
• Eligible full-length English articles focusing on SRC or scleroderma renal disease were selected.
• Data extraction and a web-based survey of the Scleroderma Clinical Trials Consortium members were utilized to capture definitions.

Main Results:

• A total of 415 papers were included, yielding 40 distinct SRC definitions from 36 studies, 9 reviews, and 2 editorials.
• Significant heterogeneity was observed in the criteria used to define SRC.
• Commonly reported criteria included new-onset hypertension, acute kidney injury, hypertensive encephalopathy, seizures, microangiopathic hemolytic anemia, and kidney biopsy findings.

Conclusions:

• SRC definitions are inconsistently applied across studies, with a minority of studies providing explicit criteria.
• The heterogeneity in SRC definitions necessitates the urgent development of a consensus definition.
• A standardized definition will facilitate consistent data collection and advance the understanding of SRC.