PO:12:186 | Clinical, functional, and microvascular features in the early diagnosis and management of pulmonary hypertension in systemic sclerosis: the importance of multimodal screening strategies

Irene Carlino¹, Stefania Bisogno¹, Alberto Palazzuoli², Luigi Gennari¹ | ¹U.O.C. Reumatologia, Dipartimento di Scienze Mediche, Chirurgiche e Neuroscienze, Università di Siena, A.O.U. Senese; ²Azienda Sanitaria Locale, Novara, Italy

Background. Pulmonary hypertension (PH) is one of the most severe and potentially life-threatening complications of systemic sclerosis (SSc), with a major impact on prognosis and patient quality of life. It is often related to microvascular dysfunction and, in some cases, to interstitial lung involvement. Early diagnosis is essential but remains challenging, as initial symptoms are frequently nonspecific and may overlap with other manifestations of the disease. In this context, the use of multimodal screening strategies combining clinical, functional, imaging, and microvascular assessments may significantly improve early identification of patients at risk.

Objectives. To assess the prevalence of PH in a cohort of patients with SSc, and to analyze its correlations with pulmonary function parameters, echocardiographic findings, and microvascular abnormalities observed through nailfold videocapillaroscopy.

Materials and Methods. We conducted a retrospective analysis of 120 patients with SSc followed at our center. Pulmonary hypertension was defined echocardiographically as a systolic pulmonary artery pressure greater than 30 mmHg. All patients underwent high-resolution computed tomography (HRCT) of the chest, pulmonary function tests, echocardiography, and nailfold videocapillaroscopy. Clinical, serological, and instrumental data were collected and analyzed. Statistical analysis included t tests, chi-square tests, and logistic regression.

Results. Twenty-seven percent of patients met criteria for PH. Subjects with PH exhibited a significant reduction in DLCO (mean 47.2 ± 13.3 vs 58.8 ± 11.8; p<0.001), a lower FVC/DLCO ratio, and signs of right ventricular dysfunction (mean TAPSE: 19.5 ± 3.8 mm vs 23.1 ± 3.1 mm; p<0.001). Capillaroscopic findings showed a significantly higher prevalence of the “late” pattern in patients with PH (62% vs 23%; p<0.01), characterized by avascular areas, giant capillaries, and loss of microvascular architecture. sPAP was inversely correlated with DLCO (r = –0.56) and positively correlated with capillaroscopic damage score (r = 0.42). A significant association between anti-centromere antibody positivity and PH was also observed (p=0.03).

Conclusions. Our findings confirm a high prevalence of PH among patients with SSc and highlight its association with impaired pulmonary function, right-heart dysfunction, and advanced microvascular damage on videocapillaroscopy. The integration of pulmonary function tests, echocardiography, and nailfold videocapillaroscopy may represent a non-invasive and effective approach for the early identification of patients at risk of developing PH. These results support the adoption of integrated, multidisciplinary screening models in clinical practice to ensure early diagnosis and monitoring of pulmonary hypertension in systemic sclerosis. Future prospective studies will be essential to confirm these data and to evaluate their long-term prognostic impact.