Leveraging Artificial Intelligence for the Diagnosis of Systemic Sclerosis Associated Pulmonary Arterial Hypertension: Opportunities, Challenges, and Future Perspectives
Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a life-threatening vascular complication of SSc, marked by high morbidity and mortality. Early diagnosis remains a major challenge due to nonspecific symptoms and the limitations of conventional tools such as echocardiography (ECHO), pulmonary function tests (PFTs), and serum biomarkers. This review evaluates the emerging role of artificial intelligence (AI), particularly machine learning (ML) and deep learning (DL), in improving the diagnostic landscape of SSc-PAH. A comprehensive literature search was conducted across PubMed, Scopus, IEEE Xplore, Embase and Google Scholar to identify studies involving AI applications in SSc, pulmonary arterial hypertension (PAH), and their intersection. Evidence indicates that AI models can assist interpretation across modalities, including heart sounds, ECGs, chest X-rays (CXRs), ECHOs, CT pulmonary angiography (CTPA), and omics-based biomarkers. While several models show encouraging diagnostic performance, their accuracy varies by dataset and modality, and most require external validation against right heart catheterization (RHC)-confirmed cohorts. Integrating multimodal data through AI frameworks may enhance early recognition and individualized risk stratification; however, these tools remain exploratory. Future work should emphasize harmonized hemodynamic definitions, transparent validation protocols, and SSc-specific datasets to ensure clinical applicability and reproducibility.