NPPB and telangiectasis: This patient had telangiectasia, negative ACA antibody, normal NT-proBNP level of 73.7 pg/mL, no right axis deviation on ECG, well-preserved WHO functional class (WHO FC) of 2, and 6MWD of 467 m, but PFT showed a very low DLCO predicted value of 44% and high FVC/DLCO ratio of 2.27, with a normal FVC percentage predicted value of 100%.