Screening for Pulmonary Hypertension in Connective Tissue Diseases: Literature Review and Multidisciplinary Consensus...

Area of Science:

• Rheumatology
• Cardiology
• Pulmonology
• Medical Screening

Background:

• Connective tissue diseases (CTDs) increase the risk of pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH).
• Early detection of PH in CTD patients is linked to improved outcomes.
• Current screening methods for PH in CTDs include imaging, biomarkers, and pulmonary function tests.

Purpose of the Study:

• To develop consensus-based recommendations and a screening algorithm for PH in patients with CTDs.
• To synthesize evidence from a systematic literature review on PH screening methods in CTDs.
• To provide guidance for early PH detection in high-risk CTD populations.

Main Methods:

• A multidisciplinary consensus group (10 rheumatologists, 4 cardiologists, 3 pulmonologists) was convened.
• A systematic literature review of the PubMed database was conducted to identify relevant studies.
• Consensus was reached based on literature data and expert opinion.

Main Results:

• 33 relevant articles were identified through the literature review.
• Significant heterogeneity was noted in study definitions, cutoff values, and patient symptom status.
• An algorithm prioritizing echocardiography for PH screening in systemic sclerosis (SSc) and SSc-overlap CTD patients was developed.

Conclusions:

• Echocardiography is recommended for screening and early detection of PH in SSc patients and those with SSc overlap features.
• Limited data prevents recommendations for asymptomatic CTD patients without SSc overlap.
• Further research is needed to establish screening guidelines for broader CTD populations.