Abstract Objective Anticentromere antibody (ACA)-positive interstitial lung disease (ILD) in systemic sclerosis (SSc) is traditionally considered less aggressive than anti-topoisomerase I (ATA)-positive ILD. However, its clinical profile and prognosis remain poorly defined. We aimed to characterise ACA-associated SSc-ILD and compare it with other serological subsets. Methods Multicentre cross-sectional observational study including 76 ACA-positive SSc-ILD patients, compared with ATA-positive (n = 54) and ACA/ATA-negative (n = 147) groups. Results ACA-positive patients developed ILD earlier than ATA-positive and ACA/ATA-negative individuals (2.5 vs. 5.6 and 3.5 years; p = 0.389). Compared with the other groups, they had higher pFVC (96.3% vs. 83.5% and 86.9%; p = 0.013), lower pFEV1/pFVC ratio (91.6% vs. 99.2% and 98.8%; p = 0.004), and better functional capacity, with fewer NYHA class III–IV patients (14.5% vs. 42.6% and 32%; p = 0.017). They also showed lower pKCO (71.7% vs. 85.1% and 77.4%; p = 0.008) and higher pFVC/pDLCO ratio (1.8 vs. 1.4 and 1.5; p = 0.016), suggesting indolent microvascular damage and increased pulmonary vascular resistance. Differences in pDLCO were not significant (60.2% vs. 60.9% and 65.7%; p = 0.769). ILD worsening (ATS criteria) occurred in 14.5% of ACA-positive patients. No significant differences were found in antifibrotic indication or advanced interventions. However, 2.6% underwent lung transplantation (0% ATA-positive, 1.4% ACA/ATA-negative) and 2.6% received autologous haematopoietic stem cell transplantation (0% and 3.4%, respectively). Overall survival analysis revealed no significant differences across the three groups (p = 0.164), although ACA/ATA-negative patients showed better survival than ACA-positive patients (p = 0.041; HR 0.498, p = 0.045). Conclusion ACA-positive SSc-ILD does not seem to have a more favourable long-term prognosis compared with other serological subsets.
Valera-Ribera et al. (Thu,) studied this question.