Patients with SSc–PAH with myocardial oedema had a higher ECV [37 (30–38) vs. 29 (27–32)%, P = 0.037], NT-proBNP [1600 (970–3600) vs. 210 (120–570) ng/L, P = 0.006], and estimated RVSP [63 (53–87) vs. 38 (33–61) mmHg, P = 0.043] and a worse DLCO [35 (20–37) vs. 44 (33–55)%, P = 0.039] than patients with SSc–PAH without oedema (n = 15). Here, NPPB is linked to edema.