Patients with PH-ILD had a bad prognosis at 3 years, which was worse than patients with PAH, even if the statistical significance was nearly missed (3-year survival of 47% vs 71%; P = .07). In the study by Condliffe et al,5 the 3-year survival was 30% in patients with PH-ILD and 47% in patients with isolated PAH (P = .02). In the study by Mathai et al,3 the 3-year survival was 39% in patients with PH-ILD and 64% in patients with isolated PAH (P < .01). Altogether, these results confirm that patients with SSc with PH-ILD are different from patients with PAH, not only in terms of SSc manifestations but also in terms of prognosis. This is also consistent with the bad prognosis of PH in patients with IPF. In this latter disease, as well as COPD, mPAP appears to be an independent risk factor for mortality8‐10,23,24: the higher the mPAP, the poorer the prognosis. In the same way, PVR is an important prognostic factor in IPF.25 Usually, in ILD or in COPD, an mPAP > 35 mm Hg is considered to be too high to be entirely explained by the lung disease, delineating a subgroup of patients with mild to severe PH sometimes called “out of proportion” PH. This out of proportion PH is associated with a worse prognosis. Recent guidelines suggest that these latter patients should be referred to expert centers and enrolled in clinical trials targeting PAH-specific drug therapy, since there is currently no approved drug in this indication.26 Interestingly, we did not find any unexpected difference in baseline characteristics between patients with SSc with PH-ILD according to the level of mPAP. Stroke volume index was also similar, suggesting that the right ventricle function was comparable between these two subgroups. Dlco was lower in the moderate to severe PH-ILD but the difference was weak. Survival between mild PH-ILD and moderate to severe (which could be called out of proportion) PH-ILD in SSc was not different, with a similarly poor prognosis. mPAP was, therefore, not a prognostic factor in PH-ILD in our study, nor was it in the study by Mathai et al.3 Therefore, separating patients with PH-ILD according to the mPAP to assess the prognosis like in other ILD is not evidence-based in SSc. Conversely, Dlco and pericardial effusion were the sole prognostic factors in this subgroup both in univariate and multivariate analysis. Only one study had previously focused on the prognostic factors in PH-ILD in a smaller number of patients.3 Dlco was also found to be one of the two prognostic factors found in the patients with PH-ILD in the latter study.3 In the setting of PH-ILD, the decrease in Dlco is likely the result of progressive pulmonary vascular remodeling or a reduction in lung volume due to ILD. However, a lower Dlco may also be related to the development of pulmonary venoocclusive-like disease, which seems frequent in SSc as suggested by two recent histologic studies,27‐32 and which could be difficult to diagnose in the context of ILD. Pericardial effusion is an usual prognostic factor in idiopathic PAH33 but has never been studied before in the setting of PH-ILD in SSc. Interestingly, baseline WHO functional class as well as hemodynamics did not appear as significant prognostic factors in our ILD-PH population. Conversely, in the Mathai et al3 study, PVR appeared to be associated with the prognosis with a weak statistical significance (P = .04).