The anti-U3RNP antibody is associated with PHT [7,57,58], myositis [57,58], and gastrointestinal involvement [56], whereas the anti-U11/U12 RNP antibody is linked to ILD [59], PHT [18], and gastrointestinal involvement [59]. Here, RNU12-2P is linked to pulmonary hypertension, primary, 1.