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Forced vital capacity <70% is the most powerful predictor of death in patients with systemic sclerosis-linked pulmonary hypertension.
A novel retrospective study highlights the critical role of a routine lung function test—forced vital capacity (FVC)—in predicting survival among patients with systemic sclerosis/scleroderma (rare autoimmune rheumatic disease)-associated pulmonary hypertension, a condition known for its high mortality risk.
Researchers analyzed 53 patients with pulmonary hypertension due to systemic sclerosis across two Italian scleroderma centers since 2015. Patients were categorized based on high-resolution computed tomography (HRCT) findings and pulmonary function:
The overall 5-year survival rate was 66.7%. Patients with PH-ILD had notably worse survival than those with PAH (Table 1).
FVC <70% emerged as a critical threshold, with survival dropping sharply to 29%, compared to 80% in those with FVC ≥70%. Receiver operating characteristic (ROC) analysis confirmed FVC as a reliable prognostic marker (area under curve = 0.713). On multivariate analysis, FVC <70% at diagnosis was the only independent predictor of mortality. Patients with PH-ILD and FVC <70% experienced the most severe decline over time, including:
The findings underscore that functional impairment—measured by FVC—may be more predictive of outcomes than radiological extent of lung disease on HRCT scans. A baseline FVC below 70% serves as a strong warning sign, identifying high-risk patients who may benefit from closer monitoring and earlier, more aggressive treatment.
Therapeutic Advances in Musculoskeletal Disease
Forced vital capacity as a survival predictor in systemic sclerosis-associated pulmonary hypertension
Claudia Iannone et al.
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