Scleroderma clinical trials consortium classification criteria for systemic sclerosis heart involvement

Area of Science:

• Cardiology
• Rheumatology
• Medical Classification

Background:

• Systemic sclerosis heart involvement (SHI) is a poorly understood condition with high mortality.
• Existing methods for diagnosing SHI lack standardization, hindering research and clinical management.
• The Scleroderma Clinical Trials Consortium (SCTC) Cardiac Working Group aimed to develop robust classification criteria.

Purpose of the Study:

• To develop and validate classification criteria for Systemic Sclerosis Heart Involvement (SHI).
• To enable systematic investigation and standardized classification of patients with SHI.
• To improve the understanding and management of cardiac manifestations in Systemic Sclerosis.

Main Methods:

• An international, inter-disciplinary group utilized consensus methods and literature review.
• Provisional criteria were refined through continuous consensus exercises and a discrete choice experiment.
• Sensitivity and specificity were tested in an independent cohort of 168 SHI cases and controls.

Main Results:

• The final SCTC SHI Classification Criteria include 23 items covering cardiac fibrosis, inflammation, arrhythmias, and vasculopathy.
• Diagnosis requires abnormalities across multiple domains; no single item is pathognomonic.
• A score of ≥11 achieved 78% sensitivity and 96% specificity for SHI, with an AUC of 0.87.

Conclusions:

• The developed SCTC SHI Classification Criteria exhibit high sensitivity and specificity.
• These criteria provide a standardized approach for classifying SHI patients in research studies.
• Application of these criteria will facilitate future investigations into this significant disease manifestation.